0

Abstract: Case Reports

Chest. 2009;136(4_MeetingAbstracts):1S. doi:10.1378/chest.136.4_MeetingAbstracts.1S-d
FREE TO VIEW

INTRODUCTION:  Mustard gas (sulfur mustard-SM) is an alkylating chemical warfare agent widely used in previous conflicts and most recently in the Iran-Iraq war. Stockpiles of SM exist in several countries and represent a continued threat as a potential terrorist weapon. Exposure can result in cutaneous, ophthalmic, and pulmonary toxicity along with systemic effects to include bone marrow toxicity.

CASE PRESENTATION:  We present a case series of three US soldiers who were inadvertently exposed to SM vapor and liquid after detonating a cache of Iraqi munitions. These soldiers were exposed for approximately 5 minutes and noted cutaneous burns/blisters, conjunctival irritation, and dyspnea within 8 hours of exposure. All had detectable levels of SM exposure by biomarker levels. Pulmonary function tests were normal with a mildly depressed DLCO and negative methacholine challenge. Plain radiographs, laryngoscopy, and oxygen saturation were all normal. Subjective dyspnea on exertion out of proportion to objective findings has persisted for 10 months post-exposure. Empiric treatment with inhaled corticosteroids and bronchodilators for airway reactivity failed to provide benefit.

DISCUSSIONS:  Our case series outlines the evaluation and management of 3 soldiers exposed to mustard gas during Operation Iraqi Freedom, and reviews the long-term complications of SM exposure. Previous literature has reported delayed pulmonary effects to include COPD, bronchiectasis, asthma, airway narrowing, and pulmonary fibrosis. Delayed pulmonary complications are typically exacerbated over time in regards to both severity and frequency.

CONCLUSION:  In most cases of mild sulfur mustard pulmonary toxicity, much of the patients’ evaluation is unrevealing with regards to a pathophysiologic explanation for persistent respiratory complaints. Often, imaging with high-resolution CT offers the best objective data for long-term follow-up of pulmonary complications. We present the evaluation and management of 3 cases of mild pulmonary SM toxicity from Operation Iraqi Freedom and review the literature on delayed pulmonary effects of SM.

DISCLOSURE:  Jacob Collen, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):1S. doi:10.1378/chest.136.4_MeetingAbstracts.1S-e
FREE TO VIEW

INTRODUCTION:  We report a case of late airway anastomotic dehiscence associated with migratory staple and sirolimus use following bilateral lung transplantation.

CASE PRESENTATION:  A 49-year-old male who underwent lung transplant for alpha-1 antitrypsin deficiency in 2004 now presented with 3-month history of dry cough associated with exertional dyspnea and a decline in his forced expiratory volume in one second. He had been taking sirolimus, cyclosporine, mycophenolate mofetil for immunosuppression following an episode of graft rejection 3 years ago. He was also on atorvastatin for hyperlipidemia for many years. Physical examination was unremarkable. High-resolution chest computed tomography showed extra-luminal gas inferomedial to the right mainstem bronchus and patchy consolidation (Figure 1). Flexible bronchoscopy showed a protruding surgical staple at the clean-looking anastomotic line confirming partial airway dehiscence Shennib grade 2a (Figure 2). An uncovered ultraflex metallic stent was placed in the bronchus intermedius. Pseudomonas aeruginosa was subsequently cultured from the lung tissue obtained from a transbronchial lung biopsy. Histology showed no evidence of cellular rejection but instead chronic inflammatory infiltrate of the airway with increased eosinophils and organizing pneumonia consistent with an infection or drug reaction. Cultures from bronchoalveolar lavage and lung tissue were negative for viral or fungal infection. He received empirical ciprofloxacin, inhaled colistimethate, tobramycin and voriconazole. Sirolimus and atorvastatin were discontinued. A repeat bronchoscopy 4 weeks later showed an intact & patent stent at the bronchus intermedius with well-formed granulation tissue. A follow-up CT showed improving infiltrate.

DISCUSSIONS:  Our case is unusual in that the airway dehiscence occurred 4 years after lung transplant raising the possibility that migration of surgical staple into the anastomotic site precipitated by chronic lung infection with concurrent sirolimus and statin use playing a supportive role; had led to this late-onset occurrence. Staple line breakdown from chronic low grade fungal infection such as aspergillosis due to local tissue invasion had been reported. Given the indolent clinical picture and CT characteristics of the lung lesions, our patient may have had chronic necrotizing aspergillosis with superimposed pseudomonas infection causing staple migration to the anastomotic site and precipitating a partial dehiscence. Early bronchial anastomotic dehiscence (< 3 months postoperative) secondary to defective airway healing has been associated with the use of sirolimus-based immunotherapy regime during immediate post lung transplant period (1). Sirolimus, a macrocyclic lactone derived from rapamycin had been shown to interfere with granulation tissue formation and wound healing by inhibiting proliferation of fibroblasts, endothelial and smooth muscle cells. Concurrent use of HMG-CoA reductase inhibitors (atorvastatin) had also been demonstrated to interact synergistically with sirolimus in inducing fibroblast apoptosis and wound breakdown in animal experimental models. Patient with anastomotic dehiscence may present with dyspnea, a drop in spirometry readings or in advanced stage, sepsis if complicated by infection. Flexible bronchoscope remains the gold standard diagnostic tool in demonstrating early mucosal ulceration, necrosis or unraveling of sutures at the anastomotic sites. In this case, a late detection would have led to metalloptysis and worsening dehiscence. Temporary placement of uncovered self-expanding metallic stents has become an accepted treatment for airway dehiscence in recent years by establishing airway patency and promoting granulation tissue formation (2). It is removed when the dehiscence heals around 6 to 8 weeks time.

CONCLUSION:  Late anastomotic dehiscence following lung transplant can arise from migratory staple and sirolimus use. Early recognition through bronchoscopic surveillance, adequate treatment of underlying infection and prompt endobronchial intervention are paramount to a favorable outcome.

DISCLOSURE:  Ai-Ping Chua, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):1S-f-2S. doi:10.1378/chest.136.4_MeetingAbstracts.1S-f
FREE TO VIEW

INTRODUCTION:  Early-onset symptomatic emphysema has been described to occur in susceptible individuals under age 50. Herein, we describe a 48-year-old smoker with severe panlobular emphysema occurring in association with multicentric reticulohistiocytosis, a papular, infiltrative cutaneous eruption that is a member of the proliferative histiocytic (CD68) spectrum of diseases.

CASE PRESENTATION:  A 48-year-old female with a 30 pack-year history of smoking and no prior cardiopulmonary disease was referred for evaluation of dyspnea. The patient had a history of an extensive, rapidly progressive, erythematous, coalescing papular eruption affecting predominantly her hands, arms, back, chest and thighs consistent with multicentric reticulohistiocytosis and confirmed by skin biopsy. Her dyspnea had been present for 2 years and had been progressive. She also reported dry coughing with no history of fever, weight loss, hemoptysis, chest pain, or night sweats. Medical therapy for her proliferative histiocytic condition included etanercept, methotrexate, and prednisone with clearance of the skin lesions at the time of her pulmonary evaluation. She did not have any family members with lung disease. There were no HIV risk factors nor history of illicit drug use. Vital signs were normal and lung auscultation revealed diminished air entry in a symmetric fashion with no wheeze. Skin findings upon initial presentation are shown in Figure 1. The remainder of the examination was unremarkable. Complete blood count and comprehensive metabolic panel were within normal range. Chest radiography revealed hyperinflation of both lungs with emphysematous changes in the upper lungs, and prominence of the central pulmonary arteries. Spirometry showed a FEV1/FVC ratio of 39, with a FEV1 of 35% of predicted. The diffusing capacity adjusted for hemoglobin was 37%. Computed tomography of the chest (Figure 2) showed extensive emphysematous changes predominantly in the upper lungs. Serum alpha-1-antitrypsin (A1AT) level was within normal limits (166 mg/dL, reference 100 - 190 mg/dL); A1AT genotyping did not detect either S or Z alleles. HIV test was negative.

DISCUSSIONS:  The diagnosis of early-onset symptomatic emphysema is established in patients younger than 50 years. Several conditions have been associated with premature emphysema, including A1AT deficiency, HIV infection, cocaine and other inhalational drugs, primary connective tissue disorders such as cutis laxa, hypocomplemetic urticarial vasculitis syndrome, and histiocytic proliferative syndromes such as multicentric reticulohistiocytosis (1). Multicentric reticulohistiocytosis is a rare, non-Langerhans cell histiocytosis characterized by a cutaneous papulo-nodular eruption, often with destructive arthritis (2). Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis (2). The disease is associated with malignancy in approximately 25–30% of cases. The diagnosis is confirmed by the presence of oncocytic (“ground-glass”) histiocytes and multinucleated giant cells on histopathology of the cutaneous lesions or the synovial membrane (2).

CONCLUSION:  Histiocytic proliferative syndromes such as multicentric reticulohistiocytosis must be considered in the differential diagnosis of emphysema occurring in young adult smokers.

DISCLOSURE:  Rodrigo Cartin-Ceba, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):2S. doi:10.1378/chest.136.4_MeetingAbstracts.2S-d
FREE TO VIEW

INTRODUCTION:  Rosai-Dorfman Disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare disease caused by a benign proliferation of lymphocytes. Most patients present with painless adenopathy, most commonly in the cervical lymph nodes, but other sites have been described as well. Extra-nodal involvement is rare but most commonly affects the head and neck. Laryngeal and tracheal involvement has rarely been reported in the literature. We report a case of near complete obstruction of the distal trachea treated which was effectively treated with electrocautery snare, Xomed® microdebrider, and argon plasma coagulation.

CASE PRESENTATION:  A 77 year-old female with a medical history significant for breast cancer 7 years prior presented to her primary care physician with complaint of a persistent cough for 6 weeks. She denied any shortness of breath, dyspnea on exertion, fevers, chills, night sweats, or weight loss. Computerized tomography (CT) of her chest revealed a 2 cm intraluminal tracheal mass arising from the anterior wall of the trachea with associated mediastinal and hilar adenopathy. This was thought to represent recurrence of her breast cancer. She underwent flexible bronchoscopy revealing a spherical shaped mass with near complete occlusion of her tracheal lumen 3 centimeters above the carina. An electrocautery snare could only remove 50% of the mass. She was admitted to the hospital for definitive therapy. She underwent rigid bronchoscopy and a Xomed® microdebrider was used to debulk the tumor followed by argon plasma coagulation for hemostasis. The tumor was shaved down to the residual tracheal wall and it was noted intra-operatively that the tumor was invading the anterior and lateral walls of the trachea. Biopsy specimens taken at the time of the procedure were consistent with extra-nodal RDD. A repeat CT scan two months post-procedure revealed recurrence of the mass in the trachea with 25% intraluminal obstruction present despite a 3 month course of prednisone. She completed one course of 20 Gy external beam radiotherapy and follow up imaging reveals that current therapy has stabilized the growth of the tumor.

DISCUSSIONS:  RDD is a rare lymphoproliferative disorder which usually causes painless adenopathy, but may present with symptoms caused by extra-nodal involvement. Most cases follow a relatively benign course with waxing and waning symptoms and do not require therapy. Treatment options described in the literature include corticosteroids, chemotherapy, low-dose radiation therapy, and surgical debridement. These treatments are usually only necessary with vital organ involvement or airway compromise. She was effectively treated with multi-modality therapy, including bronchoscopy and external beam radiation.

CONCLUSION:  Central airway obstruction due to RDD is exceedingly rare, with only seven previous cases reported in the literature. Five of those cases were subglottic or laryngeal with extrinsic compression. We describe a case with an exophytic distal tracheal mass, which to our knowledge has not been described in the literature previously. This case is also unique in that the mean age of diagnosis is twenty years, with our patient presenting in her seventies. We successfully treated the obstruction with microdebridement, argon plasma coagulation, electrocautery and external beam radiation.

DISCLOSURE:  Keith Goulet, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):2S-e-3S. doi:10.1378/chest.136.4_MeetingAbstracts.2S-e
FREE TO VIEW

INTRODUCTION:  Spontaneous pneumomediastinum is an uncommon problem which is rarely recurrent. The etiology is idiopathic in most patients. This case illustrates an unusual cause of this problem that may merit consideration in these patients.

CASE PRESENTATION:  A 28-year-old woman presented to the emergency room with acute “puffiness” of her neck and face which appeared after she fell and struck her neck against her car. She reported multiple prior episodes of pneumomediastinum and subcutaneous emphysema which were typically preceded by minor trauma, paroxysms of coughing or hiccups. Her past surgical history was non-contributory. She was a lifelong non-smoker but had a history of opiate abuse. On examination the patient had puffiness and palpable crepitus in the chest, neck and face. The remainder of her physical examination was unremarkable. A chest radiograph showed subcutaneous air and pneunomediastinum without a coexisting pneumothorax. No pulmonary parenchymal pathology was noted. Shortly after admission the patient developed bradycardia. Subsequent computed tomography (CT) imaging confirmed the soft tissue emphysema and pneumomediastinum. The CT images also demonstrated a distal tracheal diverticulum and air dissecting along both carotid arteries –the presumed cause of her bradycardia. An esophagogram showed no contrast leakage. After stabilization in the ICU, the patient was referred for thoracic surgery evaluation. A preoperative fiber-optic bronchoscopy (FOB) revealed multiple, dynamic diverticulae of varying sizes protruding from the distal tracheal and proximal mainstem bronchial walls. Several large, well-defined diverticulae were obliterated using cautery and fibrin glue with good short-term results.

DISCUSSIONS:  Pneumomediastinum –defined as air in the mediastinum –usually occurs secondary to leakage of air into the mediastinal cavity due to a traumatic injury to the chest wall, airway or esophagus. Such trauma may be iatrogenic (thoracic surgery, chest tube placement, dental procedures, tracheostomy placement). While pneumomediastinum may also occur with pulmonary diseases (necrotizing pneumonia, obstructive lung disease, malignancy), pneumothorax is usually seen in such cases. Less often, pneumomediastinum may occur in the absence of a predisposing condition: in this case it is referred to as “spontaneous” pneumomediastinum. Spontaneous pneumomediastinum is considered a relatively benign condition and needs to be differentiated from secondary pneumomediastinum which is often associated with a grave prognosis. The diagnosis of spontaneous pneumomediastinum rests on ruling out causes of secondary pneumomediastinum like esophageal perforation, pleuropulmonary diseases or trauma. Evaluation includes a detailed history, physical examination, chest radiography and a contrast esophagogram (barium swallow). A significant number of patients with spontaneous pneumomediastinum have a history of asthma, cough or emesis. This suggests a role for increased intrathoracic pressure in the pathogenesis of spontaneous pneumomediastinum. Such patients most likely have an unknown anatomic defect that, in the presence of increased intrathoracic pressure, leads to the formation of pneumomediastinum. Tracheobronchial diverticular disease, as illustrated by this case, may be one such possible abnormality. CT scans of the chest and neck coupled with routine FOB may identify such cases of tracheal diverticulae. As illustrated by this case tracheal reconstruction or snare resection of diverticulae should be considered in patients with recurrent disease or in those who develop life-threatening complications.

CONCLUSION:  Tracheal diverticulae may constitute an under-recognized cause of secondary pneumomediastinum and should be evaluated in patients with “spontaneous” pneumomediastinum.

DISCLOSURE:  Manu Kaushik, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):3S. doi:10.1378/chest.08-2354
FREE TO VIEW

INTRODUCTION:  Tracheostenosis is a relatively uncommon complication of many benign and malignant conditions. Airway stents are often used in benign central airway obstruction. As these patients outlive the expected stent life, previously unseen stent related complications are now rising. Silicone stents are preferable in benign condition as it can be removed easily. We report the first case of a new complication of Rüsch Y stent where the posterior wall was disintegrated. During our extensive search we did not find any similar report in the literature.

CASE PRESENTATION:  In August 2005, 45 year old male developed severe angioedema requiring intubation followed by tracheostomy due to prolonged airway compromise. A month later, he was admitted with severe dyspnea and stridor. Bronchoscopic examination revealed 90% tracheostenosis, about 6 mm in diameter and 2.5 cm from carina. He was treated with Argon plasma coagulation (APC) resection of stenotic area. In the following months he required several APC resections, cryotherapy and balloon dilation to relieve symptoms. Because of the recurrent nature, we decided to place a Dumon silicone stent in February 2006. Although he was decanulated immediately after stent placement, later it migrated 3 times requiring bronchoscopic repositioning and eventual stent removal within a month. We decided to place a Y stent to prevent migration. Dynamic Rüsch Y stent was chosen because it mimics normal trachea and has improved secretion clearance. Patient improved clinically and remained symptom free for 3 years. He underwent 7 follow-up bronchoscopies between April, 2006 and February 2009. Except some granulation tissue at proximal end of the stent no other difficulties were encountered. During last bronchoscopy in February 2009, a defect was found on the posterior wall of the stent with protrusion of tracheal wall during respiration. This was not seen during previous bronchoscopy on February 2008. Because of compromised integrity of the stent and to avoid any ongoing tracheal injury, the stent was removed with rigid bronchoscopy. The stent had foul smell and had a 1.5 cm × 2.0 cm area of posterior wall disintegration.

DISCUSSIONS:  In July 2005, Food and Drug Administration (FDA) issued notification against the use of metallic stent in benign conditions. Common complications include stent migration, granulation tissue formation, mucus plugging, infection or stent fracture. Silicone stents are an appropriate choice in benign tracheostenosis. Hour-glass shaped stents or Y stents are less likely to migrate. Rüsch Y stent was chosen as we expected the stent to be in place for a prolonged duration. Because of the difficulty and expertise required for Rüsch Y stent placement, it is not used in most centers, hence long term experience with this stent is limited. Review on 135 cases with Rüsch Y stent from 1997 did not report any similar complication. Possible etiology for this unusual complication could be: 1) Any damage to the stent during placement with Freitag forceps that might have increased in size over time: no such injury was found during immediate post-placement and 7 follow-up bronchoscopies over two years; 2) Chronic cough or even normal breathing causing repeated shear stress to the pliable posterior wall: possible in our case; 3) Infection weakening the stent wall: possible in our case as stent was foul smelling; 4) Chronic Acid reflux: there were no clinical or bronchoscopic evidence such as arytenoid or tracheal inflammation seen in our patient; 5) Smoking related damage to the stent: our patient is non-smoker.

CONCLUSION:  Disintegration of Rüsch Y Stent is an unusual complication. Although Rüsch Y stent helps with secretion clearance, it may not be an ideal choice in benign cases with an expected duration of more than two years.

DISCLOSURE:  Kalpesh Patel, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: stent , trachea
Chest. 2009;136(4_MeetingAbstracts):3S-d-4S. doi:10.1378/chest.136.4_MeetingAbstracts.3S-d
FREE TO VIEW

INTRODUCTION:  Unilateral absence of the pulmonary artery (UAPA) is a rare congenital disorder with variable clinical presentation. We report a case of right pulmonary artery agenesis discovered in an adult patient presenting with uncontrolled asthma secondary to recurrent bronchopulmonary infections.

CASE PRESENTATION:  A 53-year-old non-smoker woman was referred to pulmonary clinic because of uncontrolled asthma and frequent respiratory infections. She also described symptoms consistent with gastroesophageal reflux disease (GERD) and rhinitis. Her past medical history included hypertension.At the initial evaluation, the patient was in no acute distress, afebrile and her vital signs were stable. Her physical examination revealed decreased breath sounds with rhonchi in the right lower lung zone. There was no edema, cyanosis or clubbing. Laboratory data was significant for mild anemia. An allergy skin test was positive for dust mite, cats and multiple grasses. Inhaled corticosteroids and long acting beta-agonists were added as well as proton pump inhibitors, nasal steroids and anti-histamines. On subsequent visits, the patient reported improvement in her GERD and rhinitis symptoms but her asthma control was still not optimal.The pulmonary function test showed FEV1/FVC ratio of 83, FEV1 of 1.43L (65 % of predicted), FVC of 1.72L (64% of predicted), total lung capacity (TLC) of 67 % of predicted and a diffusion capacity for carbon monoxide (DLCO) of 75 % of predicted. Plain chest radiograph showed a loss of volume of the right lung, an increased density in the right lower lung zone with ipsilateral displacement of the mediastinum. A contrast-enhanced computed tomography of the thorax confirmed the radiograph findings and revealed absence of the right pulmonary artery. The patient refused further invasive workup. Her symptoms improved after a course of oral antibiotics and after optimizing her asthma therapy.

DISCUSSIONS:  Congenital UAPA is a rare anomaly that may occur in isolation but most frequently is accompanied by cardiovascular malformations. The prevalence of isolated UAPA is estimated to be around 1:200,000 individuals.The main embryologic defect is an involution of the proximal 6th aortic arch of the affected side leading to an absence of the proximal pulmonary artery.UAPA is twice more common on the right side, however left-sided agenesis is frequently associated with life-threatening cardiovascular malformations warranting early diagnosis and surgical repair. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms. Majority of patients are identified incidentally. Symptoms can be unmasked by factors such as pregnancy or high altitude. Recurrent pulmonary infections, decreased exercise tolerance, shortness of breath on exertion and hemoptysis are common manifestations. Pulmonary hypertension is present in 20- 44 % of patients. Typical chest radiographic findings are mediastinal displacement, hemidiaphragm elevation with volume loss of the affected lung, absent hilar shadow and hyperinflation of the contralateral lung. Multiple conditions like Swyer-James-MacLeod’s syndrome, compensatory emphysema and pulmonary thromboembolic disease can have similar radiographic appearance. Contrast-enhanced computed tomography of the thorax usually confirm the absence of the affected pulmonary artery. Echocardiography can establish the diagnosis and exclude any cardiac or major vessels abnormalities. Angiography remains the gold standard for the diagnosis of pulmonary artery agenesis, it is rarely performed unless embolization is indicated for massive hemoptysis. The treatment of UAPA in adults depends upon the clinical presentation. Revascularization of the absent artery is recommended when pulmonary hypertension is present. Pneumonectomy or lobectomy for recurrent hemoptysis or intractable pulmonary infections can be performed.

CONCLUSION:  Clinicians should be aware of the possibility of undiagnosed cases of UAPA presenting with recurrent respiratory infections. A chest radiograph is usually the initial investigation that suggests the diagnosis which can be confirmed by CT scanning. Angiography is reserved for patients requiring embolization or revascularization surgery.

DISCLOSURE:  Hafez Hayek, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):4S. doi:10.1378/chest.09-0087
FREE TO VIEW

INTRODUCTION:  Palpitations are best defined as an intermittent “thumping,” “pounding,” or “fluttering” sensation in the chest. It is a common symptom that is seen in primary care, emergency rooms and specialty clinics. There is a wide differential diagnosis for this including endocrine eg. thyrotoxicosis, psychatric eg. anxiety and cardiovascular eg. atrial fibrillation causes. In fact, most patients with palpitations do not have serious arrhythmias or underlying structural heart disease.

CASE PRESENTATION:  A 66 year-old man developed palpitations in the morning which he initially ignored. When the palpitations persisted for 4 hours, he presented to the emergency room. He had no present or prior history of chest pain, palpitations, shortness of breath or syncope. He had a past history of type 2 diabetes mellitus for 3 years on Januvia and hypertension for 4 years on hydrochlorothiazide. Family history was non-contributory.On exam, he was normotensive (136/83 mm Hg) and tachycardic (168 beats/min). Cardiac, respiratory, thyroid and abdominal exams were normal. Electrocardiography revealed a wide complex tachycardia with a left bundle branch block pattern. In view of this, he was started on intravenous amiodarone and transferred to us for further care.He converted to sinus rhythm with left bundle branch block at a heart rate of around 80 beats/min. Overnight, his troponin T levels trended upwards from 0.13 - 0.20 - 0.24 (normal < 0.01) ng/mL. Therefore he was started on intravenous heparin and sent for a coronary angiogram the next day which revealed an 80% mid-right coronary artery lesion to which a stent was delivered. He also had an echocardiogram that revealed an ejection fraction of 20% with evidence of left ventricular non-compaction.Therefore, he was sent for an electrophysiological study that revealed an atrio-ventricular nodal reentrant tachycardia which was ablated successfully. An implantable cardioverter-defibrillator was then placed for primary prophylaxis of sudden death, he was started on Warfarin for thromboembolism prophylaxis and heart failure medications and dismissed subsequently. On follow up at six months, he remains asymptomatic and active.

DISCUSSIONS:  Isolated left ventricular non-compaction (LVNC) syndrome is a rare genetic primary cardiomyopathy thought to arise from intra-uterine arrest of normal myocardial compaction. It is defined by the presence of prominent trabeculations, communication between the ventricular cavity and the ventricular wall through trabeculations, presence of a two layer wall structure and end-systolic ratio of non-compacted to compacted (NC/C) myocardium of > 2:1 in the absence of coexisting concomitant congenital cardiac anomalies. The exact prevalence of this condition is unclear. LVNC has been associated with heart failure, cardioembolic events and ventricular tachyarrhythmias. Our patient had a wide complex tachycardia which sparked concern for ventricular tachycardia but this was actually an atrio-ventricular nodal reentrant tachycardia. To the best of the author’s knowledge, this is the only case in reported literature of LVNC presenting as palpitations and supraventricular tachycardia.

CONCLUSION:  Isolated left ventricular non-compaction syndrome is a rare primary cardiomyopathy which may present in a variety of clinical scenarios. Echocardiography plays a pivotal role in the diagnosis. Management of the condition should focus on standard heart failure therapy, thromboembolism prophylaxis and family screening.

DISCLOSURE:  Gautam Kumar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):4S. doi:10.1378/chest.136.4_MeetingAbstracts.4S-e
FREE TO VIEW

INTRODUCTION:  Severe pulmonary hypertension (PAH) generally has a poor prognosis. This case report illustrates an apparent reversible cause of PAH due to vitamin C deficiency.

CASE PRESENTATION:  A seventy-three year old female never having smoked complained of worsening exertional dyspnea over three months. She could walk no more than 20 feet (6 meters) and her exercise tolerance was categorized as NYHA class III.On presentation, her vital signs were within normal limits. Physical examination, however, demonstrated a severely emaciated appearance, weighing 43.1kg (BMI 16.4). Cardiac auscultation showed grade 3/6 systolic murmur. The lungs were clear to auscultation. She had multiple dark, pigmented, hyperkeratotic skin lesions with excoriations covering all her extremities and trunk. Oral examination showed gingivitis, dental decay, and severe halitosis. The remaining examination was insignificant. The initial concern was acute coronary syndrome and pulmonary embolism. Her coronary angiogram showed normal coronary arteries. Pulmonary embolism was ruled out with V/Q scan. An echocardiogram showed normal left ventricular ejection fraction, normal wall motion abnormality, moderate tricuspid regurgitation, and severe PAH. Right-side heart catheterization revealed pulmonary artery pressure (PAP) 73/32 mmHg (mean 48), cardiac index (CI) 1.2 L/min, normal pulmonary capillary wedge pressure, without evidence of right heart failure. PAP was improved to 51/18 mmHg (mean 30), and CI to 1.2 L/min after adenosine infusion. Pulmonary function test (PFT) showed a mildly restrictive ventilation defect: FVC of 2.81 L (65%), FEV1 of 2.12 L (63%), FEV1/FVC ratio of 96%, with DLCO at 46% of the predicted level. A chest CT scan showed biapical parenchymal scarring with mild emphysematous changes.On further questioning, the patient believed she was sensitive to multiple foods and could not tolerate any citrus. Her vitamin C level was undetectably low. Given the appearance of her skin lesion, the clinical team considered the possibility of a vasculitis versus scurvy. Her anti-nuclear-antigen was mildly elevated, but all other vasculitis associated rheumatologic markers or SCL-70 antibodies were negative. A skin punch biopsy showed no features of vasculitis; instead showing perivascular mononuclear cell infiltrate with extravasated red blood cells, which supported a diagnosis of scurvy.The patient was discharged with Nifedipine 10mg three times a day and vitamin C supplementation. At a three month follow-up, she no longer had dyspnea or exercise limitation. Her weight had increased to 49.0 kg (BMI 18.6). The vitamin C level rose above the normal reference range and the skin lesions had resolved. Her echocardiogram showed a markedly improved estimated PAP of 35 mmHg above the right atrium pressure. A repeat chest CT showed resolution of the parenchymal changes. Complete PFT demonstrated a normalization of lung volumes, though DLCO remained reduced at 45% of the predicted level.

DISCUSSIONS:  Vitamin C deficiency is rarely seen in developed countries. Vitamin C is essential to cross-link propeptides in collagen synthesis. Purpura due to vessel wall fragility is a well known manifestation of scurvy. It would be reasonable to assume vitamin C deficiency affected the vascular elasticity of the pulmonary arterial wall, however, its effect on pulmonary artery endothelial cells or smooth muscle is not clearly understood. Similar to our patient, rare cases of pseudovasculitis, secondary to vitamin C deficiency in anorexia nervosa, have been reported (1). Our case is unique in that, first, given we had ruled out other potential etiologies, her severe PAH was likely due to vitamin C deficiency. Second, her PAH responded dramatically to low dose vasodilators; and thirdly, her PAH was symptomatically improved and objectively reversed after vitamin C replacement.

CONCLUSION:  We report the case of PAH secondary to vitamin C deficiency, which was successfully reversed after vitamin replacement.

DISCLOSURE:  Soichiro Nagamatsu, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):5S. doi:10.1378/chest.136.4_MeetingAbstracts.5S-d
FREE TO VIEW

INTRODUCTION:  Transient pulmonary hypertension mediated by vasoconstriction and hypoxia with over perfusion of certain regions of the pulmonary vascular bed is thought to contribute to the development of high altitude pulmonary edema (HAPE). Patients with pulmonary vascular abnormalities demonstrate an increased susceptibility to HAPE. We report a case of a woman who presented with fibrosing mediastinitis with complete occlusion of the left pulmonary artery and unilateral HAPE.

CASE PRESENTATION:  A 40 year-old woman with polycystic kidney disease, hypertension and mitral valve prolapse, flew to Colorado on vacation. She is originally from Indiana but move to Houston 10 years ago. Upon arrival to Denver, the patient experienced headache and nausea but continued to travel 5 hours by car to Aspen. Her symptoms persisted. She experienced an episode of dizziness, without loss of consciousness, lasting about 10 seconds. The following day, the patient went up to an elevation of 3000 meter. She became dyspneic at rest and had productive cough of pinkish sputum. The patient returned to the hotel where her symptoms improved, however her headache and nausea persisted, promptly returning to Houston. Her symptoms of nausea and dyspnea on exertion persisted, and sought medical attention. On admission, she was hypoxemic with an oxygen saturation of 94% on 4 liters at rest, and crackles in the right mid lung zone. Ultrasound was negative for DVT. Chest radiograph revealed a right lung alveolar infiltrate with left hilar calcification. Chest CT PA demonstrated abrupt termination of the left pulmonary artery at the level of the left hilum and densely calcified left hilar lymph nodes. No definite left pulmonary artery branches were identified. Alveolar opacities were noted throughout the right lung. Echocardiography was normal without evidence of pulmonary hypertension. A V/Q scan demonstrated lack of perfusion to the left lung with normal ventilation and a matched decreased perfusion and ventilation in the right upper lung without PE in the right lung.

DISCUSSIONS:  This is a unique case of unilateral right-sided HAPE due to complete occlusion of the left pulmonary artery secondary to fibrosing mediastinitis probably caused by histoplasmosis. In 1997, a case report of a 5-year-old boy who developed unilateral HAPE with left pulmonary artery agenesis. More recently in 2009, a 35 year-old female with worsening headaches, shortness of breath and chest pain was found to have mediastinal fibrosis causing occlusion of the left pulmonary artery without HAPE. HAPE is a life-threatening form of non-cardiogenic pulmonary edema that occurs in healthy individuals at altitudes above 2500m, however, some cases have been reported at lower altitudes. HAPE is characterized by dyspnea secondary to non-cardiogenic pulmonary edema, frequently preceded by symptoms of headache, nausea and vomiting. The improvement in our patient’s symptoms on descent to lower altitude in the absence of therapeutic intervention, suggests a diagnosis of HAPE. Chronic forms of mediastinal fibrosis (MF) include Idiopathic Proliferative Fibrosing Mediastinitis (IPFM) and fibrosing mediastinitis most commonly due to Histoplasma capsulatum. MF is one of the more severe, late complications of infection with histoplasmosis. Post histoplasmosis MF is characterized by calcified fibrosis centered in lymph nodes, which may occlude major vessels or airways. With IPMF, the mass does not usually contain calcifications. Radiographic presence of calcification (greater than 1 cm) carries high specificity of FM due to histoplasmosis. Our patient’s calcification in the left hila was 2.2 cm × 1.2 cm of diameter. The mass in IPMF is usually bulkier and more diffuse than that of MF related to histoplasmosis.

CONCLUSION:  This is a unique case of unilateral HAPE due to complete occlusion of the pulmonary artery secondary to granulomatous fibrosing mediastinitis.

DISCLOSURE:  Johan van Jaarsveld, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):5S. doi:10.1378/chest.136.4_MeetingAbstracts.5S-e
FREE TO VIEW

INTRODUCTION:  Mycoplasma pneumoniae is one of the most common respiratory pathogens. The most common presentation is community acquired pneumonia in which it accounts for more than 20% of cases, but is responsible for upper respiratory illness as well. It is usually considered a noninvasive organism. Extrapulmonary disease especially in terms of cardiac involvement is unusual. Mycoplasma pneumoniae-associated pericarditis is one such manifestation that can lead to life threatening pericardial effusion and tamponade. Pericarditis as a complication has been reported in 4.5 % of all serologically diagnosed cases of acute mycoplasmal infection. The lack of feasible culture methods and under appreciation of the pathogen’s ability to cause invasive disease is responsible for reduced number of diagnosed Mycoplasma pneumoniae related complications.

CASE PRESENTATION:  A previously healthy 53 year old Hispanic male presented with 3 days duration sharp epigastric pain associated with nausea and vomiting. Physical examination was found to be significant for tachycardia, jugular venous distension, muffled heart sounds, pulsus paradoxus, diminished breath sounds at lung bases and ascitis. Chest x-ray demonstrated enlarged cardiac silhouette with bilateral pleural effusions. EKG showed sinus tachycardia and electrical alternans. Bedside Echocardiogram revealed large pericardial effusion with evidence of tamponade. Subsequently, patient was taken to operating room for pericardial window but the patient went into cardiopulmonary arrest requiring cardiopulmonary resuscitation, following which open sternotomy was performed. Twenty five hundred cc of bloody fluid was drained which was sent for analysis. Analysis showed mostly blood with white blood cells and rare atypical cells, possibly reactive mesothelial origin. Pericardial biopsy showed dense fibrocollagenous tissue and adipose tissue with inflammatory cell infiltration. No neoplastic or granulomatous process was noted. Work up for autoimmune etiology was negative. Serology demonstrated significantly elevated mycoplasma pneumoniae IgG titers > 600. There was no evidence of any other causative agents in repeated stains, cultures or pathological examination. Hospital stay was further complicated with upper gastrointestinal bleed which was managed conservatively, seizures and deep vein thrombosis with pulmonary embolism requiring Greenfield filter insertion. Patient was discharged home in stable condition on azithromycin and antiseizure medication. On subsequent outpatient follow-ups patient remains asymptomatic and has been doing well.

DISCUSSIONS:  Mycoplasma pneumoniae in the repiratory tract has been suggested to reach the pericardium through the bloodstream, the bronchiolar lymphatics or by direct seeding, when the pleural-pericardial barrier is breached by a tumor or surgery. Mycoplasma pericarditis may result in pronounced morbidity unless appropriate antibiotic therapy is administered. Some cases diagnosed as idiopathic pericarditis could have a mycoplasmal etiology. Large pericardial effusions, especially in immunocompromised patients or those with previous cardiac surgery are more likely to be suspicious of mycoplasma associated pericarditis. Serology is the mainstay of laboratory diagnosis. IgM is a reliable indicator of recent Mycoplasma pneumoniae infection, but since this antibody is produced less frequently during reinfection, a negative result could be expected in patients over the age of 45 years, as in our case. The culture of mycoplasma species usually requires 1–2 weeks, thus limiting the role of this modality in guiding diagnostic and therapeutic decisions. When available, PCR, which can be done rapidly and has a high specificity, may be helpful, especially when combined with serology.

CONCLUSION:  As appropriate treatment is significant for the outcome of mycoplasma associated pericarditis, serologic testing for the mycoplasma species should be part of the routine workup for pericarditis of unknown cause.

DISCLOSURE:  Sharad Bajaj, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):5S-f-6S. doi:10.1378/chest.136.4_MeetingAbstracts.5S-f
FREE TO VIEW

INTRODUCTION:  Pulmonary hypertension (PH) is a rare but potentially lethal complication of plasma cell dyscrasia (PCD), with polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M) and skin changes (S) known as POEMS syndrome. Cyclic AMP response element binding protein (CREB) levels are decreased in smooth muscle cells (SMCs) in remodeled pulmonary arteries from animals with pulmonary hypertension and atherogenic systemic arteries and cardiomyocytes from hypertensive individuals. It is thought that direct phosphorylation, proteolysis and intracellular localization are key mechanisms regulating CREB content and activity in SMCs. Cell culture experiments showed that proteosome inhibitors can restore cellular CREB content.1 Moreover, Bortezomib, a proteosome inhibitor, is an active and approved therapy for PCD.2.

CASE PRESENTATION:  A 64 year old white male with past medical history of coronary artery disease post CABG was in his usual state of health until three months prior to presentation when he developed significant shortness of breath on minimal exertion with ascites and lower extremity edema. He went from being an active working individual to almost completely bedbound. He had poor appetite and lost 20 pounds despite his volume overload. He denied any chest pain or symptoms suggestive of cardiac ischemia. Upon medical evaluation he was found to have WHO Class III CHF symptoms. Echocardiogram was done that showed normal left ventricular ejection fraction of 60% and pulmonary hypertension with PA systolic pressure of 65mmHg. Subsequently, left and right heart catheterizations with vasodilator challenge were performed that confirmed his pulmonary hypertension. Lab work-up revealed elevated transaminases, ESR, BNP, and proteinurea with M-spike on serum protein electrophoresis. After extensive diagnostic work-up which included liver, bone marrow, right heart and kidney biopsies, he was found to have PCD (15% plasma cells, IgG lambda) with POEMS like syndrome in addition to pulmonary hypertension, secondary right heart failure, and membranoproliferative glomerulonephritis. He was started on Ambrisetan, endothelin receptor antagonist, for his PH and Bortezomib, proteosome inhibitor, for his PCD. Patient’s dyspnea got significantly better after cycle#1 despite experiencing side-effects from his PH medications which had to be held for a while and subsequently improved back to his baseline by cycle#4. By cycle#2, he was found to be in near complete remission with complete normalization of his bone marrow and serum electrophoresis by the end of therapy. By cycle#3 his Raynaud’s syndrome was significantly better with less dysesthesia which completely resolved by cycle#6. His edema almost complete resolved by the end of the therapy. His PCD has been controlled for almost 16 months so far. He is currently on minimal dose of Lasix with minimal dyspnea on exertion and back to his active life.

DISCUSSIONS:  Although the improvement in PH symptoms can be due to PH and PCD directed therapies, the fact that PH medications were on hold when early symptom improvement was reported attributes such effect to Bortezomib. Moreover, the early and brisk response in PH symptoms agrues for an additional faster pathway than the mere PCD cytoreduction and ensuing secondary cytokine changes.

CONCLUSION:  This case not only demonstrates for the first time the potential therapeutic activity of proteosome inhibitors in POEMS associated PH, but also serves as an in vivo experiment that supports an underlying etiologic role for proteosomal degradation of CREB in SMCs. Further exploration of the role of proteosome inhibition in such disease, is warranted.

DISCLOSURE:  Shantanu Saraswat, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):6S. doi:10.1378/chest.136.4_MeetingAbstracts.6S-c
FREE TO VIEW

INTRODUCTION:  A 24-year-old pregnant woman was admitted at 33 weeks-gestation due to dyspnea of two-week duration. She had a mild dry cough, denied chest pain, fever, hemoptysis or night sweats. She had no joint pains, rashes, anorexia, abdominal pain, nausea or vomiting.

CASE PRESENTATION:  The patient was complaint with regular antenatal care and she was treated for Staphylococcus aureus UTI two-weeks prior to admission. Gestational history revealed multiple spontaneous abortions due to cervical incompetence and she had prophylactic cerclage this pregnancy. She denied toxic habits or travel history; her tuberculin test was negative six months earlier. On initial evaluation she was tachycardic, normotensive and afebrile. Her respiratory rate was 28 breaths per minute and her oxygen saturation was 95% in ambient air. There was no jugular venous distension. Rales were heard at lung bases. The abdomen was soft, gravid, and not tender. No skin lesions were noted.Initial laboratory results are shown in table 1.Chest-x-ray on admission showed bilateral diffuse nodular infiltrates (Figure 1). She was transferred to ICU for acute hypoxic respiratory failure and treated with antibiotics for community acquired pneumonia and suspected infective endocarditis. Her respiratory status worsened requiring high FiO2. Intravenous steroids were initiated for fetal maturity. On day three of hospitalization she developed worsening infiltrates and was electively intubated followed by hemoptysis. Fiberoptic Bronchoscopy (FOB) revealed diffuse alveolar hemorrhage (DAH). (Table 2) Elective caesarian section with delivery of viable fetus was performed. Her hospital course was complicated by ARDS, septic shock and multiple organ failure. Work up for vasculitis was negative. Beta HCG declined post delivery. Cytology and microbiology of bronchoalveolar lavage specimens was negative. Echocardiogram showed no vegetations and normal heart function. Open lung biopsy could not be done due to high oxygen and PEEP requirements. Patient continued to have DAH, refractory ARDS and shock and expired on day 13. Autopsy revealed metastatic choriocarcinoma to the lungs. Placenta revealed a microscopic focus of Choriocarcinoma.

DISCUSSIONS:  ARDS in a pregnant patient is a diagnostic challenge. This can be classified as causes unaffected by pregnancy (pneumonias, sepsis, acute pancreatitis), modified by pregnancy ( pyelonephritis, aspiration) and unique to pregnancy (obstetric hemorrhage, severe preeclampsia, chorioamnionitis, endometritis, trophoblastic embolism and amniotic fluid embolism 1. Choriocarcinoma is a rare tumor with an incidence of 1 in 25,000 to 40,000 pregnancies. It may develop after an abortion, a term or preterm pregnancy, an ectopic pregnancy, or a hydatidiform mole. It is estimated that the lesion is preceded by a normal pregnancy in 22.5% of the cases. Choriocarcinoma associated with a viable pregnancy is rare but has been reported in literature. This condition is suspected in gravid patients with pulmonary lesions and failure of beta HCG to decline after delivery. DAH has been described in primary pulmonary choriocarcinoma 2 but not in metastatic choriocarcinoma.In our case initial diagnostic consideration for ARDS included infection and vasculitis and extensive work up was negative. Placental gross exam was normal and beta HCG trended down. Our case represents a rare manifestation of choriocarcinoma without vaginal bleeding presenting with ARDS and DAH with declining beta HCG after delivery.

CONCLUSION:  Metastatic choriocarcinoma should be considered in the differential of ARDS in pregnant patients with DAH. A careful review of placental tissue and consideration to lung biopsy should be done in pregnant patients with unclear lung pathology.

DISCLOSURE:  Swapna Muppuri, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):6S-d-7S. doi:10.1378/chest.136.4_MeetingAbstracts.6S-d
FREE TO VIEW

INTRODUCTION:  Abnormal elevation in serum lipids is extremely common. If severe, hyperlipidemia can have serious medical consequences. Acute pancreatitis is an acute potentially life-threatening complication of severe hypertriglyceridemia. Additionally, elevated levels of triglycerides can interfere with clinical laboratory testing. We present a case of a woman presenting with diabetic ketoacidosis and severe hypertriglyceridemia treated with plasmapheresis when conventional treatments failed to significantly reduce her triglyceridemia.

CASE PRESENTATION:  A 63 year old woman with chronic, untreated diabetes presented to the emergency department with a 1 week history of a tender mass at the apex of her scalp. She was hypertensive and tachycardic and required incision and drainage for a scalp abscess. Initial chemistry panel returned uninterpretable due to severe lipemia. A lipid panel revealed a cholesterol level of 1,051 mg/dL and triglyceride level of 10,132 mg/dL. Laboratory after bench ultracentrifugation at another institution revealed a glucose of 559 mg/dl, a bicarbonate of 12 mmol/L, and an anion gap metabolic acidosis consistent with diabetic ketoacidosis (DKA). The lipemia-associated interference in laboratory studies made treatment of her DKA-related electrolyte abnormalities extremely difficult. Her hypertriglyceridemia was initially managed with insulin, antilipidemic medications, and heparin but remained significantly elevated delaying labs and treatment. Plasmapheresis was initiated and serum triglycerides rapidly decreased from 7,501 mg/dL to 1,745 mg/dL in less than 24 hours. (Figure 1).

DISCUSSIONS:  Serum triglyceride concentrations exceeding 1000 mg/dL (11 mmol/L) are considered severely elevated and pose a high risk for development of acute pancreatitis. Severe lipemia also interferes with clinical laboratory testing by three mechanisms: (1) turbidity resulting in light scattering, (2) increase in the non-aqueous phase of the sample, or (3 partitioning between the polar and non-polar phases. Circulating lipid particles, namely VLDL and chylomicrons, cause turbidity by bending light away from its original path due to scatter, reflectance, or absorption. Additionally RBC hemolysis is enhanced by lipemia, due to erythrocyte fragility. Because electrolytes are in the aqueous phase of blood, lipids may dilute their concentration. Lipemia can be minimized by reducing the interfering lipid, ultracentrifuging the sample (100,000g) or using polymers to precipitate the lipids out of the sample. The process of ultracentrifuging a sample may produce excess heat and cause error due to release of intracellular potassium. Plasmapheresis is one method to rapidly remove excess lipids. Case reports demonstrate successful use of plasmapheresis for hypertriglyceridemia associated with acute pancreatitis, gestational hypertriglyceridemia, induction chemotherapy associated hypertriglyceridemia, pancreatitis with ARDS due to hypertriglyceridemia, protease inhibitor-induced hypertriglyceridemia, familial hypercholesterolemia, and, like our case, poorly controlled diabetes with severe hypertriglyceridemia.

CONCLUSION:  Plasmapheresis is a safe, rapid and effective intervention for the emergent management of severe hypertriglyceridemia that results in significant reductions in triglycerides allowing timely measurement of serum electrolytes. Consider plasmapheresis when lipemia interferes with clinical labs and conventional treatments such as heparin, insulin, antihyperlipidemic drugs and fat-free parenteral nutrition are ineffective in acutely lowering triglyceride levels.

DISCLOSURE:  Gilbert Seda, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):7S. doi:10.1378/chest.136.4_MeetingAbstracts.7S-d
FREE TO VIEW

INTRODUCTION:  Pulmonary Arteriovenous Malformations (AVMs) are often associated with chronic hypoxemia due to right-to-left shunting. We present a case of a patient with Hereditary Hemorrhagic Telangiectasia (HHT) and arteriovenous malformations (AVMs) who developed severe hypoxemia on mechanical ventilation. We suggest precautions in the management of patients with right-to-left intrapulmonary shunting when initiating mechanical ventilation, and management options for worsening hypoxemia.

CASE PRESENTATION:  An 84-year-old Caucasian female with a history of HHT and pulmonary AVMs on home oxygen at night presented to the emergency department with a severe headache. CT angiogram of the brain revealed two AVMs. Her arterial blood gas (ABG) on a non-rebreather mask with fraction of inspired oxygen (FIO2) 100% revealed a pH of 7.39, a pCO2 of 33, and a PaO2 of 63, and she was admitted to the ICU for hypoxemic respiratory failure, presumably related to increased right-to-left intrapulmonary shunting as no other clear precipitants were identified. Spiral CT of the chest did not reveal pulmonary embolism or significant air-space opacities, but demonstrated multiple AVMs, with the largest measuring 3.2 cm x 1.8 cm in the right upper lobe. There were no prior CT scans for comparison. Contrast echocardiography confirmed right-to-left intrapulmonary shunting with normal right ventricular function and an estimated pulmonary artery pressure of 36 mm Hg, and percutaneous embolization of the pulmonary AVMs was scheduled. Given worsening hypoxemia (SpO2 of 80–82% with 100% FIO2) in spite of empiric diuresis, and the anticipated need for transport to the Angiography department and peri-procedure sedation, she was intubated after receiving Etomidate and Rocuronium. Time-cycled pressure-controlled ventilation was initiated with peak inspiratory pressure at 20 cm H20;PEEP at 5 cm H20; inspiratory time at 1.2 seconds, respiratory rate at 10, and FIO2 at 100%, and her SpO2 dropped to 60% with ABG values as follows: pH, 7.47; pCO2, 33;PaO2, 25; and SaO2, 55%. The left lateral decubitus position was used to minimize shunting in the right upper lobe AVM with little improvement. After her paralytic agent wore off, a change to pressure support ventilation 10 cm H20 without PEEP improved the SpO2 to 78–80%. Emergent embolization of her largest AVM improved the SpO2 to 98% immediately. She was discharged to home after 7 days without evidence of hypoxemic brain injury on 2 liters per minute of oxygen, with SpO2 94–96%.

DISCUSSIONS:  Positive pressure ventilation likely worsened hypoxemia in this case by increasing pulmonary capillary resistance and redistributing blood flow to the AVMs, thereby worsening right-to-left shunting. Some case reports have described worsening oxygenation with mechanical ventilation and increased PEEP in patients with HHT and right-to-left shunting (1, 2), but there are no general precautions and recommendations for the ventilator management of patients with right-to-left intrapulmonary shunting and hypoxemic respiratory failure. After our experience with this patient, and a review of the literature, we offer the following suggestions: 1) Avoid mechanical ventilation unless absolutely necessary. 2) Avoid long-acting paralytics during intubation to allow spontaneous breathing through the endotracheal tube. 3) Minimize the mean airway pressure if positive pressure ventilation is necessary. 4) Positional changes, such as the decubitus position with the AVM side up, can be considered to decrease shunting but may not be helpful. 5) Consider emergent embolization of AVMs.

CONCLUSION:  Positive-pressure mechanical ventilation may worsen hypoxemia in patients with HHT and intrapulmonary right-to-left shunting, and clinicians should be aware of management options for severe hypoxemia.

DISCLOSURE:  Yasuhiro Norisue, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):7S-e-8S. doi:10.1378/chest.136.4_MeetingAbstracts.7S-e
FREE TO VIEW

INTRODUCTION:  We report a case of pseudomembranous tracheobronchitis causing central airway obstruction in a previously healthy 48-year-old man with severe septic shock and acute respiratory distress syndrome from Streptococcous pyogenes (Group A Strep).

CASE PRESENTATION:  A 48-year-old white male presented to the Emergency Department with a three-day history of sore throat, fever, and shortness of breath. On physical examination, the patient was toxic appearing. His rectal temperature was 35.5 degrees Celsius (95.7 degrees Fahrenheit), blood pressure was 94/53 mm Hg, pulse was 143 beats per minute, respirations were 30 breaths per minute, and oxygen saturation was 89% on room air. Initial examination was significant for diffuse erythema of the head and neck with prominent cervical lymphadenopathy, bilateral proptosis, tachycardia, diffuse inspiratory and expiratory wheezing, and dusky appearing extremities. Laboratory data were significant for neutropenia, thrombocytopenia, elevated creatinine, elevated liver enzymes, and lactic acidosis. Chest x-ray showed a patchy alveolar pattern. Admission computed tomography (CT) scan of the chest showed dense parenchymal consolidation of the lower lobes and scattered alveolar opacities of the upper lobes. The patient was intubated for impending respiratory failure, admitted to the intensive care unit, and treated for septic shock. Within 24 hours, blood cultures were positive for Streptococcus pyogenes. By day four, physical examination was notable for severe desquamation of the extremities. On day seven, he developed worsening oxygenation and ventilation. A CT scan of the chest showed persistent alveolar opacities, parenchymal consolidation, and evidence of mucous secretions within the trachea and mainstem bronchi bilaterally (Figures 1). The patient subsequently underwent diagnostic fiberoptic bronchoscopy revealing diffuse pseudomembranes involving the majority of the trachea and bilateral mainstem bronchi (Figure 2). There was near complete obliteration of the left main bronchus. Flexible bronchoscopic forceps were unsuccessful in removing the obstructing tissue. Airway patency was re-established when cryo-adhesion therapy was utilized to remove the pseudomembranes. The histo-pathologic findings were remarkable for large amounts of mucoid material with extensive hemorrhage and acute inflammation with varying amounts of fibrin. Herpes PCR was positive, but viral inclusions were not seen on the pathological specimen. The patient ultimately recovered and was discharged home.

DISCUSSIONS:  Streptococcus pyogenes sepsis is a rare event, but the mortality is high due to a rapidly progressive course and multiorgan failure. Common manifestations of the infection include pharyngitis and cellulitis. It is also frequently reported in the obstetrics literature as puerperal infections. More serious presentations include the toxin-mediated diseases such as necrotizing fasciitis and toxic shock syndrome (TSS). (1) Pseudomembranous tracheobronchitis has been reported in association with endotracheal intubations, smoke inhalation injury, and infections such as Aspergillus spp., methicillin-resistant Staphylococcus aureus, Bacillus cereus, Corynebacterium diphtheriae, viruses and rarely inflammatory bowel disease. (2) Several of the infectious etiologies that have been associated with pseudomembranes are toxin producing organisms. The severe desquamation that our patient experienced coincided with the endotracheal mucosal sloughing. This was thought to be a consequence of toxin production by S. pyogenes. Herpes simplex is a frequent isolate from airway samples obtained during respiratory failure. In our case, no viral inclusions were noted on pathologic specimens and were likely an incidental finding.

CONCLUSION:  Our patient suffered from central airway obstruction due to pseudomembranous tracheobronchitis associated with TSS. He was successsfully treated with bedside therapeutic bronchoscopy. Upon review of the literature, this is the first reported case of pseudomembranous tracheobronchitis associated with S. pyogenes and TSS.

DISCLOSURE:  Marcia Henderson, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):8S. doi:10.1378/chest.136.4_MeetingAbstracts.8S-b
FREE TO VIEW

INTRODUCTION:  Systemic lupus erythematosus (SLE) is a systemic connective tissue disorder involving multiple organs.We report a case of SLE with Posterior reversible encephalopathy syndrome (PRES) in the setting of diffuse proliferative nephropathy and Thrombotic thrombocytopenic purpura (TTP).

CASE PRESENTATION:  34 yr old asian female with no medical history presented with rash on face, and joint pains, for 1 week. Initial vitals signs were normal. Physical examination was normal except for oral ulcers and malar rash.Initial lab work up revealed normocytic anemia, renal failure with BUN/Cr –38/1.8, ESR –58 mm. A diagnosis of SLE was made based on high levels of ANA, dsDNA, Anti Smith antibodies and low complement levels. Renal biopsy revealed stage IV diffuse proliferative glomerulonephropathy. Patient was treated with high dose steroids. Clinical course—On hospital day 4, the patient developed severe headache, blurred vision which was followed by generalized tonic clonic seizures. Patient was treated with dilantin and transferred to the MICU. MRI of head revealed multiple areas of increased T2 signal in cortex and subcortical white matter in the temporal-occipital and posterior corpus callosum without evidence of vasculitis consistent with PRES. Blood pressure was tightly controlled with maintainance of systololic BP between 120–130 mmHg with the use of nicardipine infusion. Her symptoms gradually resolved over next 7 days. Her BP was maintained in stated range with the use of labetalol and hydralazine. MRI repeated with in 1 week revealed resolution of white matter changes. During her ICU stay, the patient also developed severe anemia and thrombocytopenia. Peripheral smear showed schistocytes consistent with hemolytic anemia and platelet count < 10K. A presumptive diagnosis of TTP was made and later confirmed with low ADAMTS-13 activity and patient was treated with plasmapheresis. Hematologic indices initially improved with daily plasmapheresis, but later relaped requiring 3 courses of Rituximab. Patient continued to have worsening renal function despite treatment with steroids and cyclophosphamide requiring hemodialysis. At present patient remains on hemodialysis for end stage renal disease due to glomerulonephropathy but with normal hematologic and CNS function.

DISCUSSIONS:  This case highlights the unique association of PRES, TTP, and SLE. PRES is a clinical syndrome of headache, confusion, decreased level of consciousness, visual changes, and seizures, associated with characteristic radiological findings of posterior cerebral white matter edema. Well known risk factors for development of PRES are uncontrolled hypertension, eclampsia and immunosuppressive medications. A sudden rise in blood pressure with failure of auto-regulatory mechanisms leading to fluid extravasation has been the most accepted hypothesis for the development of PRES. Our patient did not have traditional risk factors for development of PRES. Treating clinicians must have high index of suspicion for this particular condition as prompt treatment results in rapid resolution of symptoms and neurological deficits in most cases. Treatment usually involves tight control of blood pressure and control of seizures. Plasmapheresis is an effective treatment in patients with TTP and we believe helped in the resolution of PRES. Unlike in some other forms of cerebral edema, use of steroids should be limited as it may worsen PRES.

CONCLUSION:  SLE patients presenting with neuropshychiatric symptoms are often a diagnostic dilemma. Although they are more likely to have diagnosis such as vasculitis, cerebritis, venous thrombosis, and strokes, PRES should be considered in the differential. Characteristic findings on MR imaging help differentiate PRES from other diagnosis. Tight control of BP is critical.

DISCLOSURE:  Kavan Ramachandran, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):8S-c-9S. doi:10.1378/chest.136.4_MeetingAbstracts.8S-c
FREE TO VIEW

INTRODUCTION:  Herpes simplex viral (HSV) pneumonia is a less commonly recognized cause of pneumonia in patients admitted to the Intensive Care Unit. We report a case of fatal herpes simplex pneumonia in a post thoractomy patient.

CASE PRESENTATION:  : A 67 year old woman with stage IIIa left upper lobe (LUL) squamous cell carcinoma received one cycle of neoadjuvant chemotherapy with Cisplatinum and Taxotere. The immediate post chemotherapy period was complicated by a brief episode of febrile neutropenia. She was empirically treated with broad spectrum antibiotics. Three months following neoadjuvant chemotherapy, she underwent left thoracotomy with LUL lobectomy and pericardial repair. On post operative day (POD) 3, she developed progressive hypoxemia, chest tightness and bilateral diffuse infiltrates. She was started on broad spectrum empiric antibiotic coverage with Cefepime, Vancomycin and Ciprofloxacin. On POD 4, the patient’s condition continued to worsen, and she required endotracheal intubation. Her sputum cultures were negative for bacteria and fungi. Clinically, there was no evidence of cardiogenic pulmonary edema. On POD 5, patient received stress dose steroids (Hydrocortisone 50 mg iv every six hours), which was tapered to off over next five days. On POD 6, the patient had episodes of high grade fever with worsening leucocytosis (20,000/dL) so Fluconazole had to be empirically added. Deep tracheal secretions were sent to culture, which turned out negative. The patient started improving slowly in the following days. However on POD 10, she again had episodes of high grade fever. After a sputum culture, Fluconazole and Cefepime were changed to Caspofungin and Meropenem respectively. No other sources of infections were identified. The patient also had worsening renal failure for which hemodialysis was initiated. A urine Legionella antigen was negative. Repeat sputum specimen was negative for bacteria or fungi. The patient’s A-a gradient continued to worsen and on POD 16 she required ventilatory support with airway pressure release ventilation. Her condition worsened further on POD 28, when she developed disseminated intravascular coagulation with upper gastrointestinal bleeding. On POD 31, the family decided to provide her comfort care and she was terminally extubated. A limited chest autopsy was performed. Sections of lung revealed diffuse necrotizing pneumonia. The respiratory epithelium and the alveoli were filled with atypical cells, which by immunohistochemisty were positive for HSV (both 1 and 2 types) infected cells. The cells demonstrated nuclear molding, multinucleation, and intranuclear inclusions. She also had herpetic esophagitis but no oral lesions were seen.

DISCUSSIONS:  The exact role of HSV isolation in mechanically ventilated patients—whether it is a true pathogen leading to morbidity/mortality or an asymptomatic carrier remains a matter of debate. Current guidelines do not routinely recommend testing for herpes pneumonia. However, early diagnosis by bronchoscopy and bronchial washing, can be made by identifying HSV virus itself, immunohistochemistry or isolating specific herpetic nuclear inclusion bodies in bronchoalveolar lavage. If diagnosed early, HSV pneumonia may be successfully treated with Acyclovir. The major risk factor for HSV infection in our patient was immunocompromised status after chemotherapy. In addition, Camazine et al. have suggested that thoractomy in thoracic oncology patients by itself is a significant factor for immunosuppression. She probably had reactivation of HSV in the oropharynx. Pulmonary involvement may have resulted from local extension from the oropharynx after intubation and prolonged mechanical ventilation.

CONCLUSION:  HSV pneumonia should be considered in an immunocompromised patient not responding to antibiotics and antifungal agents.

DISCLOSURE:  Ritwick Agrawal, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):9S. doi:10.1378/chest.08-2858
FREE TO VIEW

INTRODUCTION:  Phosgene (carbonyl chloride) gas is of historical interest and has important industrial applications today. Historically, it was the most lethal of World War I war gases. It is estimated that nearly 80% of the poison gas deaths during that war were caused by phosgene exposure. In the post 9/11 era, phosgene remains a potential, highly toxic chemical weapon. Additionally, phosgene poses significant risk to some workers who may be occupationally exposed. This case report demonstrates a known hazard of refrigeration workers suffering phosgene poisoning after heating chlorinated fluorocarbons (Freons).

CASE PRESENTATION:  49 year-old refrigerator technician was admitted to our intensive care unit following phosgene gas exposure. The patient reports using a welding torch while soldering a refrigerator coil which contained Freon. Following vaporization of Freon to phosgene the patient immediately noted a peculiar, pungent smelling gas. He then experienced lacrimation and a burning sensation in his mouth and throat, followed by severe dyspnea, wheeze and cough. On arrival to the emergency department the patient was dyspneic and complained of chest tightness and palpitations. The patient’s heart rate was 140 bpm, blood pressure was 110/60, oxygen saturation was 98% on a non-rebreather facemask. Physical examination revealed an obese male in moderate distress. His conjunctiva was injected. His respirations were mildly labored but clear to auscultation. The cardiovascular exam was irregularly irregular. The remainder of his exam was normal. Chemistries and complete blood count were within normal limits. An arterial blood gas revealed a pH of 7.39, pCO2 of 36 mmHg, and pO2 of 92 mmHg on a FiO2 of 1.0. The patient’s chest X-ray was normal and telemetry monitoring showed a ventricular rate of 140 bpm and atrial fibrillation. Following CDC and OSHA recommendations, appropriate measures were taken to minimize risk of poisoning hospital personnel including monitoring of the patients exhaled gas phosgene levels. This remained detectable (>1 ppm) for approximately 5 hours, after which isolation procedures were discontinued. The patient was admitted to the ICU for observation with rapid resolution of hypoxic respiratory failure and was discharged within 48 hours.

DISCUSSIONS:  This patient’s symptoms were typical of those seen after exposure to phosgene at a concentration exceeding 3 ppm. 1 The possibility of occupational exposure of refrigeration workers to phosgene has been rarely reported. Adequate purging of refrigeration pipes before welding should help to reduce this risk significantly. Phosgene is a highly toxic gas, and exposure may have a fatal outcome. Respiratory symptoms may be delayed by a latent period of several hours before acute respiratory distress syndrome (ARDS) becomes apparent. The mechanisms of lung injury have been elucidated in animal models to include acylation and lipid peroxidation. These processes result in denaturation of proteins and lipids, irreversible alterations of membrane structures, and disruption of enzyme and other cell function. 2 Treatment is primarily supportive as there is no known antidote for phosgene. The most severe cases benefit from close monitoring and positive pressure ventilation, but the role of corticosteroids and other adjuncts of care remain controversial. Specific treatment regimens for phosgene exposure and ARDS have not been investigated in human, prospective randomized controlled trials.

CONCLUSION:  Phosgene exposure is associated with significant morbidity and mortality. Physicians should be aware of the risk of phosgene exposure when heat is applied to Freon, commonly used in a variety of industries. Patients with a history of exposure should be admitted to the hospital for observation given the potential for delayed onset of respiratory failure and ARDS.

DISCLOSURE:  Daniel Kim, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: exposure , phosgene
Chest. 2009;136(4_MeetingAbstracts):9S-d-10S. doi:10.1378/chest.136.4_MeetingAbstracts.9S-d
FREE TO VIEW

INTRODUCTION:  Tracheobronchial amyloidosis is a rare disorder of unknown cause associated with the extracellular deposition of amyloid protein in a characteristic spatial structure of beta-sheet fibrils assembled into bundles. This protein stains with Congo red and shows apple-green birefringence under polarized light. The present case, which represents the nodular form of tracheobronchial amyloidosis, is the least common form of pulmonary amyloidosis with less than 20 cases reported.

CASE PRESENTATION:  An 81 year old female presented to the outpatient pulmonary clinic with shortness of breath and wheezing. She was recently hospitalized with a left lower lobe pneumonia. Her past medical history included “asthma,” pneumonia, hypertension, and acid reflux disease. She had no relevant surgical, social or family history. Her vital signs, physical examination, laboratory studies, spirometry and conventional chest x-rays were normal. Subsequent contrast enhanced chest computed tomography (CT) scan demonstrated multiple tracheal masses, left lower lobe posterior basilar collapse, and calcification of the cartilaginous tracheal rings and posterior membrane. Bronchoscopy demonstrated multiple fixed, firm, nodular hypervascular tracheal masses less than one centimeter in diameter, scattered hard irregular yellow plaques, and abnormal submucosal infiltration of the anterior main carina. There were no endobronchial abnormalities noted in the left lower lobe. [Figure 1] Forceps biopsy of the distal right tracheal lesion demonstrated eosinophilic paucicellular fibrosis with foci of calcification and congo red stain demonstrated submucosal amyloid deposits with apple-green birefringence under polarized light consistent with a diagnosis of nodular tracheobronchial amyloidosis. [Figures 2].

DISCUSSIONS:  Patients with nodular tracheobronchial amyloidosis may present with shortness of breath, exertional dyspnea, hoarseness, cough, wheezing, and hemoptysis due to endobronchial obstruction or infiltration. Occasionally, patients present with recurrent post-obstructive pneumonia or endobronchial stenosis. Patients are often incorrectly diagnosed with asthma, pneumonia, or tracheobronchitis. Our patient presented with a history of recurrent pneumonias and was initially thought to have asthma. Spirometry may be helpful in detecting proximal nodular tracheal amyloidosis with classic inspiratory and expiratory loop truncation associated with fixed upper airway disease. In tracheobronchial amyloidosis, calcification typically involves both the cartilaginous rings and the membranous portion between the tracheal rings and/or the posterior membrane. This is a distinguishing feature on chest CT from tracheobronchopathia osteochondroplastica, in which calcification is reportedly confined to the anterior cartilaginous rings. Tracheobronchial amyloidosis typically show a bronchoscopic appearance of multiple bumps or endobronchial masses with overlying normal mucosa. Histologically, tracheobronchial amyloidosis can show osseous metaplasia and dystrophic calcification in addition to submucosal fibrosis. Congo red staining should be performed to evaluate for the possibility of tracheobronchial amyloidosis. Currently, the mainstay of treatment for tracheobronchial amyloidosis is debridement of symptomatic luminal obstruction via endoscopic resection and/or Nd-YAG laser therapy. Some patients may be amenable to endoscopic debridement with airway stenting or by-pass tracheostomy. External beam radiation therapy has been reportedly successful for some patients with symptomatic luminal obstruction. Open surgical resection or tracheoplasty is an option for those with diffuse disease. Once the diagnosis is established, early consultation with an interventional pulmonologist is strongly recommended. Our patient elected continued observation with a contingency plan to seek an interventional pulmonology consultation for evaluation of Nd:YAG laser therapy.

CONCLUSION:  Patients with tracheobronchial amyloidosis may present with symptoms of dyspnea, localized wheezing, cough, hemoptysis, or recurrent pneumonias. Patients are often misdiagnosed with asthma, recurrent pneumonia or tracheobronchitis, and the true cause may not be recognized. Tracheobronchial amyloidosis can be confirmed by demonstrating the presence of submucosal amyloid deposition with Congo red staining. The mainstay of therapy is debridement of symptomatic luminal obstruction. Early referral to an interventional pulmonologist is recommended for possible endoscopic debridement and/or Nd-YAG laser therapy. Other treatment strategies include airway stenting, external beam radiation therapy, by-pass tracheostomy, or open surgical resection.

DISCLOSURE:  Krish Bhadra, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):10S. doi:10.1378/chest.136.4_MeetingAbstracts.10S-c
FREE TO VIEW

INTRODUCTION:  We report a case of endobronchial silicosis presenting with Right Middle lobe atelectasis.

CASE PRESENTATION:  78 year old white male admitted for non-STEMI. Workup showed occluded right coronary artery and a high-grade proximal LAD lesion. Echocardiogram showed moderate aortic stenosis with severely calcified leaflets.The patient had a brief smoking history and had worked in a foundry removing cooled metal from sand molds for 40 years. He had no prior PFTS. Up to his current illness he remained quite active. He did not travel or own any pets. Denied having fevers, chills, night sweats, weight loss, dyspnea, cough, hemoptysis, rashes, arthlagias, or swellingOn physical examination lung fields were clear. Cardiac exam revealed a grade 3/6 systolic ejection murmur.Chest x-ray showed linear atelectasis in the right middle lobe. (Fig 1). Figure 1. CT Chest showed a 2.9 cm right middle lobe consolidation with calcified thoracic adenopathy (Fig 2) Figure 2. On bronchoscopic examination, the orifice of the lateral subsegment of the right middle lobe was occluded as shown in the figure 3. Figure 3. Bronchial washing from the right middle lobe showed benign respiratory epithelial cells, alveolar macrophages, and chronic inflammation. Biopsy from the same region had unremarkable bronchial mucosa with single focus consistent with portion of silicotic nodule consisting of fibrosis, anthracosis, and rare linear polarizable particles consistent with silicosis. Fig 4. Figure 4. The patient underwent elective coronary artery bypass grafting and aortic valve replacement. The patient tolerated both procedures well and was successfully discharged to continue his rehabilitation at a specialized facility 11 days later.

DISCUSSIONS:  Endobronchial silicosis is a known complication from exposure to silica however, very rarely seen. Only a few case reports have been identified dating back to the 1970’s. Affected patients are typically seen in regions of the world where underground mining occur and where melted metal are forged. Pittsburgh is such a region where a large population has worked in steel mills. Silica exists in both crystalline and amorphous forms. Amorphous forms include vitreous silica and diatomite which are relatively nontoxic after inhalation. In contrast, inhaled crystalline silica is associated with a spectrum of pulmonary diseases. A patient may be asymptomatic or presents only with an abnormal chest radiograph. Symptomatic patients commonly have chronic cough and dyspnea on exertion. Our patient was asymptomatic on presentation. Pathophysiology:Silica crystals translocate through the tracheobronchial-epithelial barrier into lamina propria of the bronchial mucosa to induce local pathological alterations. Silica can be phagocytized by macrophages, which are either expelled by mucociliary escalator or migrate into the bronchial mucosa. The nodular collections of silica-laden marcrophages and fibrosis can begin to protrude into the airway causing obstruction; termed endobronchial silicosis.

CONCLUSION:  Silicosis is a common disease seen years after been exposed to Silica. Endobronchial silicosis is a rare finding in this disease. When present it is usually affecting the right middle lobe causing atelectasis and can be visualized on bronchoscopic examination.

DISCLOSURE:  Tariq Cheema, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):10S-d-11S. doi:10.1378/chest.136.4_MeetingAbstracts.10S-d
FREE TO VIEW

INTRODUCTION:  Inflammatory bowel diseases are well known for producing a wide variety of extraintestinal manifestations. Pulmonary involvement is an uncommon extraintestinal manifestation, and can affect any part of the bronchopulmonary system. We present here the case of an adolescent female with Crohn’s colitis with bronchial involvement.

CASE PRESENTATION:  A 9-year old female presented to her physician with fatigue and anemia. Following a thorough workup, ileocolonoscopy and esophagogastroduodenoscopy at that time revealed colitis only of the left side and transverse portions of her colon. Her symptoms improved with steroid therapy, but she would relapse once the steroids were stopped. Symptoms included up to 10 bowel movements a day, abdominal cramping, urgency, and weight loss. Additional medical therapy included mesalamine, methotrexate, Pentasa, 6-mercaptopurine, and granulocyte colony stimulating factor. During treatment for her intestinal symptoms, she developed a persistent cough. Computed tomography (CT) scan of the chest demonstrated reactive pulmonary nodules. Infectious workup of the nodules was unremarkable, and it was thought that the nodules represented reactivity to the inflammatory bowel disease. Subsequent gastrointestinal biopsies revealed pancolitis, and the diagnosis of Crohn’s disease was suggested. She began treatment with infliximab, and an attempt at weaning her off steroids was met with an exacerbation of her gastrointestinal (GI) symptomatology. All throughout, she continued to have a slightly productive cough that waxed and waned with her GI symptoms. Repeat CT scan of the chest demonstrated mild pericarinal and hilar adenopathy. Because of the new adenopathy and long-standing nearly-chronic immunosuppression, it was decided to once again rule out infection. Flexible bronchoscopy revealed mild laryngomalacia with inspiratory prolapse of the left arytenoid into the supraglottic fossa. The vocal cords were normal. Starting at the midtrachea and extending to the distal bronchial tree bilaterally, the mucosa was significantly edematous, erythematous, friable, and diffusely nodular. Several of the mucosal nodules measured up to 3 mm X 3 mm. These nodules partially obstructed segmental bronchi particularly bronchi to the medial basal segment of the right lower lobe, lateral basal segment of the left lower lobe, and one of the subsegmental bronchi to the superior segment bronchus right lower lobe. Diagnostic broncho-alveolar lavage from the right middle lobe segments were nondiagnostic and did not reveal infection. Multiple large biopsies were taken from the mucosal nodularities in the right bronchial tree. The biopsies showed ulceration with focal suppurative inflammation and dense tissue eosinophilic infiltrate. All stains for organisms, including acid-fast, were negative.The patient continued undergoing medical treatment for her inflammatory bowel symptoms. Subsequent attempts to reduce the steroid doses were met with further exacerbation of both her gastrointestinal and bronchopulmonary symptoms.

DISCUSSIONS:  Both chronic ulcerative colitis and Crohn’s disease are known to produce a variety of extraintestinal manifestations. Although pulmonary involvement is an uncommon manifestation of these diseases, 36% to 68% of patients with Crohn’s disease have abnormal pulmonary function. Pulmonary abnormalities can present simultaneously with active bowel disease, but can also present years after the onset of the bowel disease. The pathogenesis of pulmonary disease in inflammatory bowel disease is not known, but a common systemic mechanism affecting both the bronchial and gastrointestinal epithelium is likely responsible. It is essential to rule out infection as the main etiology for pulmonary disease in patients who are immunosuppressed. It is also possible that the drug therapy administered to treat the inflammatory bowel disease symptoms may also contribute to pulmonary pathology.

CONCLUSION:  In patients with inflammatory bowel disease, deterioration of pulmonary function and an increase in pulmonary symptoms can parallel underlying disease activity. Medications used to control gastrointestinal symptoms of irritable bowel disease generally also alleviate the extraintestinal bronchopulmonary symptoms.

DISCLOSURE:  Jess Thompson, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):11S. doi:10.1378/chest.136.4_MeetingAbstracts.11S-d
FREE TO VIEW

INTRODUCTION:  Broncholiths are calcified peribronchial lymph nodes that can erode into the bronchial lumen causing symptoms of persistent cough, hemoptysis, and recurrent pneumonia. The diagnosis is often difficult to make even by bronchoscopy due to overlying and surrounding edema. Treatment often requires surgical intervention in the form of thoracotomy or a rigid bronchoscopy. We report an interesting case where not only were we able to diagnose the condition but were also able to perform a therapeutic fiberoptic bronchoscopy.

CASE PRESENTATION:  A 60 year old female with history of COPD and recurrent pneumonias presented with complaints of cough with yellow sputum and dyspnea on exertion for five months. She was febrile and hypoxic on presentation. Examination was unremarkable except for diminished air entry and bilateral crackles. Chest radiography revealed right middle lobe and right lower lobe airspace disease with a superimposed nodule; and a prominence in the right hilar paratracheal line. Non-contrast CT scan of the chest (Graphic 1) revealed diffuse airspace disease in the right middle and lower lobe with associated mediastinal and hilar adenopathy. Given the recurrent pneumonias and 30-pack-year smoking history, the patient underwent fiberoptic bronchoscopy, which revealed a necrotic, friable mass partially occluding the right mainstem bronchus. Transbronchial biopsies of the mass were performed with multiple attempts to dislodge it as we suspected it to be a broncholith. Unfortunately, the mass could not be removed bronchoscopically; however, later that day, the patient was finally able to cough and expectorate a 1.7 × 1.0 × 0.9 cm calcified, gray-tan stone (Graphic 2). Pathology from the procedures revealed squamous metaplasia with acute and chronic inflammation. The patient was subsequently discharged on antibiotics and had a follow-up CT scan that showed complete resolution of her infiltrate. Furthermore, at 3 month follow-up, the patient denied any complaints of shortness of breath, cough, hemoptysis, or fever.

DISCUSSIONS:  Broncholithiasis is an uncommon and challenging diagnosis to make and is often discovered when clinical sequelae are prominent enough to warrant detailed investigation with bronchoscopy or a CT scan of the chest. Almost all broncholiths originate in the peribronchial lymph nodes, which calcify in response to an inflammatory process. Cough, hemoptysis and recurrent pneumonias are the usual symptoms while lithoptysis occurs rarely but is specific for broncholithiasis. In our patient with a post-obstructive pneumonia that was refractory to multiple courses of antibiotics, a diagnostic bronchoscopy was planned to rule out an endobronchial lesion. A broncholith was visualized and mobilized for the patient to subsequently expectorate the stone. Biopsy of the mucosa failed to show a concurrent underlying malignancy. Not only did she have clinical and radiological recovery but complications such as hemorrhage, airway obstruction, erosions, and fistulas were avoided.

CONCLUSION:  Broncholithiasis is a diagnosis not often thought of when a patient presents with a post-obstructive pneumonia. However, failure of typical antibiotic regimens should serve to trigger a more extensive workup for broncholithiasis including a CT scan of the chest and potential bronchoscopy. Flexible bronchoscopy can be safely utilized not only for the diagnosis but also in therapeutic stone extraction.

DISCLOSURE:  Christopher Pastor, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):11S-e-12S. doi:10.1378/chest.136.4_MeetingAbstracts.11S-e
FREE TO VIEW

INTRODUCTION:  In patients presenting with massive hemoptysis, it is often challenging to control bleeding. Uncontrolled bleeding can lead to hemodynamic instability and high mortality. We present case of a patient who presented with massive hemoptysis where fibrin sealant was effective in controlling the bleeding.

CASE PRESENTATION:  A 67 year old female with stage IV recurrent non-small cell lung cancer presented with massive hemoptysis. The patient was treated with six course of Taxol/Carboplatin two years prior to presentation, but continued to have non -respectable malignancy in the left upper lobe. Patient also had history of recurrent pneumonia in left upper lobe. She complained of sudden onset of hemoptysis and reported about 1000 cc blood loss prior to arrival to emergency department. She also gave history of chronic DVT in left leg. Her medications included Coreg, Protonix, Hydromorphone and Warfarin. On examination, patient appeared anxious. Her vital signs included: temperature 36.4 C, BP 121/76 mmHg, heart rate 103 beats/minute, and respiratory rate 20/minute. Chest examination showed normal respiratory pattern, lung auscultation showed diffuse scattered rhonchi throughout the lung fields and the cardiac evaluation was also within normal limits. Initial investigation reveled an INR of 1.07 and CBC as well as electrolytes within normal limits. She underwent emergent flexible bronchscopy. It revealed extensive hemorrhage from the left upper lobe bronchial segment. A cold saline and epinephrine solution did not stop the bleeding. Approximately 2cc of fibrin sealant (TISSEEL VH by Baxter Healthcare Corporation) was injected into the left upper lobe. Following its administration bleeding stopped. A bronchial arteriogram revealed bleeding from superior right bronchial artery adjacent to medial margin of the left upper lobe tumor. Coil embolisation was performed successfully. After three days a repeat flexible bronchoscopy was performed. The left upper lobe segments did not show further bleeding. Another fibrin sealant around 3 cc was administered into the left upper lobe segments. After two more days of recovery she was discharged from the hospital and at a follow up visit after three weeks she did not report further hemoptysis.

DISCUSSIONS:  Massive hemoptysis due to pulmonary hemorrhage is a complication of localized lung lesions. They are not only a source of hemodynamic compromise but can also lead to fatal airway obstruction and ARDS. Massive hemoptysis is classically defined as expectoration of 100–600ml of blood in 24 hours. The common etiologies include: pulmonary tuberculosis, bronchiectasis, fungal and other infections, arterio-venous malformations, cardiovascular diseases, immunologic diseases and bronchiogenic Carcinoma. The fibrin sealant has been reported to be used in neurosurgical, ophthalmic, liver, kidney and bronco pleural fistulas. The sealant contains fibrinogen and thrombin. These factors when react, undergo a cascade of events and form a fibrin clot, further stabilized by factor XIII. This clot as any other clot in the human body is not permanent is under dynamic change due to enzymes which degrade the fibrin clot. The role of the fibrin sealant is thus to provide a temporary but stable clot, or a bond and thus to assist the body in healing the lesion. In our patients use of fibrin sealant played a vital role in controlling bleeding from left upper lobe.

CONCLUSION:  Fibrin sealant was effective in management of bleeding in patient with massive hemoptysis. It can be as a bridging agent until bronchial arteriogram and coiling can be performed.

DISCLOSURE:  Chirag Pandya, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):12S. doi:10.1378/chest.136.4_MeetingAbstracts.12S-c
FREE TO VIEW

INTRODUCTION:  Lemierre’s syndrome (LS) is a severe illness caused by the anaerobic bacterium, Fusobacterium necrophorum. It originates in the oropharynx and spreads via septic thrombophlebitis of the tonsillar and internal jugular veins (IJV) to sites such as the lung, joints and bones.

CASE PRESENTATION:  An 18-year-old woman with no past medical history presented to our Emergency Department (ED) with a 3-day history of fever, pleuritic chest pain and dyspnea. She had presented twice to the ED over the past 2 weeks, with complaints of sore throat, neck pain and myalgias and was discharged home with the presumed diagnosis of a viral illness. Vital signs on admission were temperature 98.8F, blood pressure 92/50, pulse 122/min, respiratory rate 22–36/min and pulse-oximetry 84% on room air. Physical exam findings included bilateral tonsillar swelling, tender cervical lymphadenopathy and bibasilar crackles. Admission labs revealed white blood cell count 42,000/cu.mm with 25% bands, blood urea nitrogen level 34mg/dl and creatinine 4.3mg/dL. Chest radiograph was consistent with a multi-lobar pneumonia. Chest CT scan revealed bibasilar consolidation with moderate right pleural effusion and cavitating peripheral opacities suspicious for septic emboli. She was subsequently intubated in the ED due to worsening hypoxemia and admitted to the ICU. She was started on ampicillin/sulbactam, vancomycin, and vasopressors for septic shock. Bedside transthoracic echo showed no valvular vegetations. Thoracentesis of the right pleural effusion was consistent with an empyema thus a pig-tail chest tube was placed. Admission blood cultures grew Gram-negative bacilli hence piperacillin/tazobactam was started. Subsequent chest radiographs and persistently high oxygen requirements were consistent with ARDS. The patient proceeded to develop a right-sided pneumothorax. Later that day, a left-sided pneumothorax ensued requiring bilateral tube thoracostomy. Continuous renal replacement therapy (RRT) was initiated on day 4 for acute renal failure (ARF). Blood cultures revealed Fusobacterium necrophorum and antibiotics were changed to meropenem and metronidazole. CT scan and ultrasound of the IJV were both negative for thromboses. Her renal function improved and pneumothoraces resolved. The patient was extubated after 2 weeks and discharged home after a 1-month hospitalization.

DISCUSSIONS:  Lemierre’s syndrome is characterized by oropharyngeal infection, Fusobacterium septicemia, findings of metastatic infection, and occasionally evidence of IJV thrombophlebitis. It classically affects healthy young adults, with a male preponderance. It was described in 1936 by Andre Lemierre who reviewed 20 cases in young adults, of which only 2 survived. It was a common disease with high mortality in the pre-antibiotic era. There is evidence of resurgence in recent years, possibly associated with reduced use of antibiotics. LS specifically relates to Fusobacterium infection arising in the oropharynx. It may include pharyngeal inflammation with exudative tonsillitis, peritonsillar or parapharyngeal abscesses. Patients often have dysphagia, neck pain and tender cervical lymphadenopathy. Septicemia usually occurs 4–5 days after the onset of pharyngitis. Lung lesions are present in 80% of cases. Cavitations, pleural effusions, and empyema can be present. Fewer than 10% of cases require mechanical ventilation. ARDS, septic shock and acute renal failure requiring RRT are unusual. Delayed antibiotic treatment may explain the severe clinical presentation of our patient. The mainstay of therapy is long-term antibiotics and drainage of collections. A carbapenem or a penicillin/β-lactamase inhibitor combination, plus metronidazole are the antibiotics of choice. Diagnostic methods to detect IJV thrombophlebitis include CT, MRI, and ultrasound. The role of anticoagulants is controversial.

CONCLUSION:  LS is a rare, life-threatening illness which can lead to septic shock and multi-organ failure if it is not recognized early. LS should be suspected in young patients with recent pharyngitis and clinical evidence of septic emboli.

DISCLOSURE:  Joseph Mathew, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):12S. doi:10.1378/chest.136.4_MeetingAbstracts.12S-d
FREE TO VIEW

INTRODUCTION:  Periferal neuropathy has been associated with Parvovirus B19. Brachial plexus involvement has been previously described, but diaphragmatic paralysis and gastroparesis as a consequence of this infection has not been reported yet.

CASE PRESENTATION:  We present the case of a 60 year old female with new onset of unilateral diaphragmatic paralysis following a confirmed Parvovirus B19 infection. Her past medical history includes controlled hypothyroidism, GERD and asthma. She developed a 9 to 10 day illness consisting of flu-like symptoms, a maculo-papular erythematous rash that started in her lower extremities and then ascended to her neck, and persistent pain in her anterior neck and shoulders bilaterally. Laboratory work revealed an elevated erythrocyte sedimentation rate (ESR) and positive IgG and IgM Parvovirus B19 antibodies one week after the onset of symptoms. There was a known exposure to a child with confirmed Parvovirus B19 infection prior to the onset of her symptoms. Workup for other infectious or rheumatologic conditions was negative. An initial CXR obtained prior to the onset of symptoms was normal and one taken 2 weeks later for evaluation of shortness of breath revealed elevation of the left hemidiaphragm. Diaphragmatic paralysis was confirmed by fluoroscopic imaging. She also developed bloating and early satiety consistent with gastroparesis.

DISCUSSIONS:  We believe our patient suffered from a Parovirus B19 infection that resulted in a left phrenic nerve injury and hemidiaphragm elevation. This virus has previously been associated with peripheral nerve damage. Two reports describe involvement of the brachial plexus with resulting pain and weakness in the shoulder girdle, as well as sensory deficits in the upper extremities (1, 2). The relation between viral infections and nerve demyelination has also been demonstrated.Our patient had a normal chest X-ray at the onset of her viral illness and one that showed a new left-sided diaphragmatic elevation 2 months after developing an immunologic response to Parvovirus B19. Her persistent shoulder pain could be an indicator of brachial plexus involvement as well. Drug related nerve damage is unlikely since she only received ketorolac, oxycodone, levothyroxine and bronchodilators during this period. She does not drink alcohol or use intravenous drugs, her thyroid function was controlled during this episode and she is not diabetic, which makes metabolic causes unlikely.

CONCLUSION:  Given the timeframe and absence of more common causes of peripheral neuropathy, our patient’s new onset of left sided diaphragmatic paralysis and gastroparesis seems to be a consequence of a Parvovirus B19 infection.

DISCLOSURE:  Martin Karlicek, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):13S. doi:10.1378/chest.136.4_MeetingAbstracts.13S-d
FREE TO VIEW

INTRODUCTION:  Viral respiratory infections (VRI’s) are generally thought of as benign, self-limited syndromes. However, VRI’s can have severe complications, even in immunocompetent hosts. This report describes the case of an 18 year old male who suffered prolonged critical illness after parainfluenza infection.

CASE PRESENTATION:  An 18 year-old man was transfered to the intensive care unit (ICU) for respiratory failure. He had no past medical history, and had felt well until ten days before admission. He initially reported nonspecific symptoms of dry cough, fever, and malaise. On the day of admission he reported worsening dyspnea, and was brought to the emergency department. There, he was found to have severe hypoxemia and respiratory failure, as well as fevers and hypotension. He was intubated and received mechanical ventilation and fluid resuscitation. Direct fluorescent antigen testing was positive for parainfluenza virus, and sputum cultures grew methcillin-resistant staphylococcus aureus (MRSA). Over the next two days, the patient developed bilateral pneumothoraces, and chest tubes were placed. The right chest-tube had a persistent air leak. After twenty-one days the patient was liberated from mechanical ventilation. He was alert and talking with his family. Two days later he suddenly developed hypoxemia, and was found rigid with frothy secretions in his mouth. He was emergently intubated. Computerized tomography (CT) of the brain showed multiple non-specific areas of low attenuation confined to the bilateral white matter. Magnetic resonance imaging (MRI) of the brain demonstrated multifocal T2/FLAIR signal abnormalities involving the white matter, along with associated petechial hemorrhages, and no evidence of ischemia or contrast enhancement. Samples of cerebrospinal fluid (CSF) showed normal cell counts, protein, and glucose levels; stains for organisms were negative, as were cultures and other microbiologic tests for viruses. These findings supported a diagnosis of acute disseminated enchephalomyelitis (ADEM). The patient was treated with anticonvulsants and high-dose corticosteroids, and was extubated after two days. His mental status improved, and although he was weak from prolonged critical illness, he was communicative and interacting normally with his family.

DISCUSSIONS:  While VRI’s are often self-limited, this case reports a young man who developed prolonged critical illness after parainfleunza infection. This case provides an unfortunate reminder that viral respiratory infections can have uncommon but potentially devastating consequences, including bacterial pneumonia (leading to ARDS, sepsis, and bronchopleural fistula), and seizures resulting from a diffuse demyelinating lesion of the central nervous system (CNS). The relationship between respiratory viruses and co-infecting bacterial pathogens is unclear, but likely involves pathogen- and host-related variables. ADEM probably results from an auto-immune process, although direct involvement on the CNS by the virus may be possible.

CONCLUSION:  Critical care physicians and pulmonologists should be alert to the potential complications of VRI’s, including bacterial pneumonia and acute disseminated encephalomyelitis. Furthermore, important variables related to viral pathogenicity and the host response to VRI remain to be elucidated.

DISCLOSURE:  Gordon Carr, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):13S. doi:10.1378/chest.136.4_MeetingAbstracts.13S-e
FREE TO VIEW

INTRODUCTION:  Facial vein thrombophlebitis is extremely rare in the current antibiotic era. There is only one case report described in the English lanuage literature of a patient with this condition secondary to a sore on her upper lip which acted as a portal of entry for the infection. The French and Russian literature have several case reports and case series dating back to the 60’s described as complications of furuncles and carbuncles.

CASE PRESENTATION:  A 39-year-old woman was bought to the emergency department with dyspnea, chest and back pain, along with tenderness, erythema and swelling on the right side of her face. Her symptoms began 2 weeks ago, with a painful vesicular rash involving the lower right side of her face around the lips. She was diagnosed with herpes zoster and started on oral acyclovir therapy. After approximately 10 days she noticed increased swelling, erythema and tenderness at the angle of the jaw on the right spreading along her right cheek. This was followed a day later by increased shortness of breath, chest and back pain. The patient moved from Pakistan 9 years ago. She had a history of hypothyroidism on levothyroxine replacement, which was her only daily medication. She had no allergies and worked as a housewife. Physical Examination revealed a middle aged female in moderate distress. On admission her temperature was 39.1°C, heart rate was 119 beats/min, BP was 148/78 mm Hg, respiratory rate was 28 per min, and oxygen saturation was 93% on room air. Focused physical exam revealed a vesicular rash around the right side of the lips, and swelling, redness and warmth over her right cheek. Cranial nerve examination was normal. Chest auscultation and percussion were normal. Cardiac exam revealed tachycardia without murmurs. Laboratory evaluation revealed normal electrolyte and hepatic panel. The WBC count was 14.9 with a left shift. Hemoglobin and platelet counts were normal. Plain chest film shows bilateral nodular densities. CT scan of the thorax revealed multiple pulmonary nodules bilaterally, some of which were cavitary, wedge-shaped and peripheral in distribution. CT scan of the neck showed complete occlusion of the right anterior facial vein without extension into the jugular vein proper. There was surrounding edema and induration without frank abscess formation or lymphadenopathy. Patient was started on broad-spectrum antibiotics. Transthoracic echocardiogram did not show valvular vegetations. Blood cultures grew staphylococcus aureus (MRSA) sensitive to vancomycin, bactrim and clindamycin consistent with a community acquired MRSA sensitivity profile. Patient was continued on vancomycin for the next 4 weeks with significant clinical improvement. A repeat CT scan of the thorax 5 months later showed almost complete resolution of the nodular lung densities.

DISCUSSIONS:  The anterior facial vein starts just below the medial epicanthal fold bilaterally as a continuation of the angular vein. It connects with the cavernous sinus proximally and the jugular vein distally. Intection in this area if left untreated, could lead to devastating complications like cavernous sinus thrombosis. Septic embolic complication to any organ is also possible. Our patient had septic pulmonary emboli which resolved completely with antibiotic therapy. Duration of antibiotic therapy ranges from 2–6 weeks in different case reports.

CONCLUSION:  Thrombophlebitis of the anterior facial vein is rare and therefore a high index of suspicion is needed to diagnose and treat the disease early and aggressively to avoid potentially fatal complications. Most common organim is Staphylococcus Aureus and portal of entry is generally an area of damaged skin overlying the vein.

DISCLOSURE:  Ali Kanchwala, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):13S-f-14S. doi:10.1378/chest.136.4_MeetingAbstracts.13S-f
FREE TO VIEW

INTRODUCTION:  Expanding cavitary lung lesions may lead to erosion of thoracic or extrathoracic blood vessels and hemoptysis. Hemoptysis in pulmonary aspergillosis usually stems from the bronchial vasculature, and invasion of major blood vessels is more common in tuberculosis or pulmonary malignancies. We present a case of massive hemoptysis due to invasive aspergillosis eroding the subclavian artery and successful treatment with an endovascular stent.

CASE PRESENTATION:  An 82-year-old male presented with a twelve hour history of intermittent hemoptysis and pre-syncope. Admission hemoglobin level was 8.0 g/dL. He was being treated for an expanding left upper lobe cavitary lesion previously diagnosed as chronic necrotizing aspergillosis. CT scan of the chest and arteriography revealed a new pseudoaneurysm on the inferior margin of the left subclavian artery (Figure 1). The pseudoaneurysm communicated directly with the inflammatory rim of the lung cavity suggesting a direct erosion of the arterial wall. An endoluminal stent graft was placed into the subclavian artery over the pseudoaneurysm. (Figure 2). Hemoptysis completely resolved within 48 hours of the procedure.

DISCUSSIONS:  Intrathoracic vascular pseudoaneurysms may arise in a variety of necrotizing pulmonary processes that extend to involve nearby blood vessels. Most recognized is Rasmussen’s aneurysm of the pulmonary artery in cavitating pulmonary tuberculosis, a rare cause of massive hemoptysis. Vascular pseudoaneurysms from expanding aspergillosis-related lung cavities have been reported, but only one prior case report was found describing involvement of the subclavian artery. Aspergillosis-related pseudoaneurysms are almost exclusively found in severely immunocompromised patients. Our patient was only mildly immunocompromised from recent use of corticosteroids and had structurally abnormal lungs from COPD. The progression of the previously known cavitary lesion was likely a treatment failure of itraconazole due to inadequate absorption from concomitant use of a proton pump inhibitor.

CONCLUSION:  This case illustrates a rare complication from expanding pulmonary aspergillosis as an unusual cause of hemoptysis. Secondly, it emphasizes the importance of recognizing the state of mild immunosuppression as a risk factor for the development of chronic necrotizing aspergillosis and the need to initiate aggressive appropriate antifungal therapy.

DISCLOSURE:  Lorenzo Klein, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):14S. doi:10.1378/chest.136.4_MeetingAbstracts.14S-d
FREE TO VIEW

INTRODUCTION:  Solobacterium moorei is a gram-positive anaerobic bacillus isolated from human feces. Samples taken from subjects with and without halitosis analyzed by bacterial culture and direct amplification of nucleic acids indicate Solobacterium moorei is associated with halitosis which produce volatile sulfur compounds which can be toxic to tissues. This bacteria is often associated with Fusobacterium nucleatum which has been indicated as a cause of pulmonary abscesses and upper airway infections (reference 1). Reports of infection with Solobacterium moorei are rare with two studies in the literature finding S. moorei in blood cultures in a septic patient with multiple myeloma. Another recent case reported S. moorei bacteremia in a patient with acute proctitis and cervical carcinoma. A third case implicates S. moorei as one of the pathogens in thrombophlebitis and septic pulmonary embolism emphasizing the thrombogenic potential of necrobacillosis organisms (reference 2).

CASE PRESENTATION:  A 47 year old female with a history of severe COPD presented with a 45 pound weight loss and malaise over six months. She was afebrile but experienced night sweats and back pain. Chest x-ray revealed a new cavitary lesion in the left upper lobe with elevated hilum and volume loss. CT of the chest showed a 5.7 by 6 cm large multiseptated thick-walled irregular cavitary lesion in the left upper lobe surrounded by parenchymal consolidation in the apical and posterior segments with air bronchograms seen within the consolidated portion as well as severe diffuse emphysema predominantly in the upper lungs consistent with her smoking history (figure 1). PET scan showed intense hypermetabolic activity in the left upper lobe corresponding to the lesion and the infiltrative aspect of the lesion (figure 2). A bronchoscopy with brush biopsy and bronchoalveolar lavage was performed. Anaerobic cultures from the lavage revealed gram positive rods which were isolated to be Solobacterium Moorei with the following MIC: Clindamycin 8 μg/ml, Ciprofloxin 0.75 μg/ml and Metronidazole 0.125 μg/ml. Left upper lobe biopsy was consistent with rare non-caseating granulomas. The patient received a 43 day course of metronidazole however her antibiotic regimen was changed to clindamycin for 54 days after she developed peripheral neuropathy in her lower extremities. The antibiotic course was finished after the symptoms subsided and follow-up CT showed a stable cavitary lesions.

DISCUSSIONS:  Solobacterium moorei may have clinically significant pathogenic potential as indicated by this case. It can be distinguished from Eubacterium, Lactobacillus, Propionibacterium and Bifidobacterium by metabolic end products of glucose fermentation. Most of the isolates are found in the oral cavity, periodontitis sites in patients with halitosis and most are associated with oro-dental diseases such as dental abscesses or odontogenic infections. Primary foci of necrobacillosis infection outside the head and neck are rare but we are reporting a primary source case in the lung. Portal of entry of the lung infection could not be established but periodontal disease constituted the most probable origin of the infection since S. moorei is essentially found among the oral flora.

CONCLUSION:  In the past, this organism has not been recognized as being clinically significant or pathological because of the specific growth requirements and lack of identification by formal testing. Often this organism if found is one of multiple species in an infection. However this case proves the pathological and significant clinical disease manifestations of this organism.

DISCLOSURE:  Anwar Haque, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):14S-e-15S. doi:10.1378/chest.136.4_MeetingAbstracts.14S-e
FREE TO VIEW

INTRODUCTION:  Musculoskeletal involvement of tuberculosis is relatively uncommon. This is a case of disseminated tuberculosis presenting as left ankle monoarticular arthritis.

CASE PRESENTATION:  A 74-year-old woman who emigrated from Mexico 12 years ago presented with 6 months of left ankle pain and swelling. Because of limited mobility the patient had fallen and fractured her left hip. After surgical repair she was referred for further evaluation of her persistent left ankle symptoms. She had no other past medical history, and denied personal or exposure history to tuberculosis. Besides the left ankle symptoms, she also reported some weight loss and an occasional dry cough. There was no fever, chills, night sweats, rashes, or other joint or muscle pains. The physical exam was only significant for left ankle swelling, mild warmth, erythema, and tenderness to palpation and with movement of the joint. There was no leukocytosis, but calcium and alkaline phosphatase levels were elevated. Left ankle arthrocentesis produced a hazy serous fluid that contained predominantly lymphocytes and monocytes, with negative acid-fast-bacilli staining. The Quantiferon-TB Gold test was positive. Chest x-ray was abnormal, with a high-resolution computed tomography of the chest showing bilateral parenchymal nodules, areas of consolidation, and calcified lymphadenopathy. After multiple sputums were negative for acid-fast-bacilli, the patient underwent bronchoscopy with transbronchial biopsy. Empiric anti-tuberculosis therapy was also initiated. Since the bronchoalveolar lavage fluid and biopsy specimen were also stain-negative, yet suspicion for disseminated tuberculosis remained high, the patient underwent left ankle arthroscopy for synovial and bone biopsy. The Ziehl-Neelsen stain of the synovial biopsy sample was negative, but the Auramine-rhodamine stain was positive. One of the induced sputum samples started growing acid-fast bacilli at 11 days, with speciation confirming growth of Mycobacterium tuberculosis. The synovial fluid from the ankle arthroscopy also started growing acid-fast bacilli at 13 days.

DISCUSSIONS:  Musculoskeletal tuberculosis is relatively uncommon, constituting 1–3% of tuberculous infections. Any bone, joint, or bursa can be involved, but 70% of musculoskeletal tuberculosis occurs in the spine, hips, or knees. During mycobacteremia of the primary infection, the tuberculosis bacilli spread to bone or joint spaces either hematogenously or via lymphatic spread. Reactivation of the disease can later lead to musculoskeletal tuberculosis. The diagnosis is often delayed because of the indolent course and low clinical suspicion. Spinal tuberculosis, or Pott’s disease, constitutes 40–50% of musculoskeletal tuberculosis. In endemic countries this disease occurs in older children and young adults, but in developed countries it is more commonly seen in older adults. Thoracic spine is involved in approximately 50% of the cases, with 25% of cases occurring each in lumbar and sacral spine. Extra-axial tuberculosis usually favors the weight-bearing joints, such as the hips and knees. There is usually only monoarticular involvement, with complaints of slowly progressive pain, swelling, and loss of function. Constitutional symptoms and active pulmonary disease are only seen in a minority of patients, and only 50% of these patients have chest x-ray abnormalities suggestive of tuberculosis. Synovial fluid analysis only has a sensitivity of approximately 79%. Acid-fast-bacilli stains are positive only in a minority of patients, and there are mixed results on the use of DNA amplification studies. Synovial or bone biopsy and culture are often needed for definitive diagnosis. The treatment of musculoskeletal tuberculosis is the same as the treatment of pulmonary tuberculosis. If treatment is initiated early the patient can often recover full joint function.

CONCLUSION:  The diagnosis of musculoskeletal tuberculosis is clinically challenging. Work-up can be extensive, and a high clinical suspicion is needed.

DISCLOSURE:  C Lee, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):15S. doi:10.1378/chest.136.4_MeetingAbstracts.15S-d
FREE TO VIEW

INTRODUCTION:  Kartagener’s syndrome (KS) is a variant of primary ciliary dyskinesia (PCD). It is an autosomal recessive disease characterized by situs inversus, sinusitis and bronchiectasis. Patients with KS develop chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. We present a case of KS with lung abscess caused by Pseudomonas aeruginosa (P. aeruginosa), Mycobacterium avium intracellulare complex (MAC) and Nocardia species (spp).

CASE PRESENTATION:  A 38-year-old white male with established KS presented to the outpatient pulmonary clinic for hemoptysis for nine months and left sided pleuritic chest pain of two weeks duration. He complained of mild dyspnea and anorexia but denied constitutional symptoms. He had received multiple courses of antibiotics and oral steroids over the last 9 months for worsening cough and productive sputum production. The patient was non-adherent with airway clearance measures. A carpenter by profession he had remained a non-smoker all his life. On physical examination enlarged nasal turbinates were observed and bibasilar crackles heard on lung auscultation. Pulmonary function testing was consistent with obstructive ventilatory impairment (FEV1 = 2.52 L (57%) and FEV1/FVC = 55%). A computerized tomography (CT) scan of the chest demonstrated a large left middle lobe cavitary lesion with an air-fluid level and background bronchiectasis with thoraco-abdominal situs inversus. Worsening infiltrates, bronchiectasis, lymphadenopathy and a small left pleural effusion were also noticed. The patient underwent a bronchoscopy with bronchoalveolar lavage (BAL) and a protected specimen bronchial brush. P. aeruginosa, E.coli, Nocardia spp. and MAC were cultures from the specimens. The patient was treated with multiple antimicrobials (see table) with resolution of the abscess. He continues to be on treatment for Nocardia spp. and MAC.

DISCUSSIONS:  KS is a rare genetic disorder. Patients with KS develop respiratory infections due to ciliary dysfunction and stasis of secretions in the respiratory tract. A systematic study of the microbiology of airway secretions in KS is lacking. Common infectious organisms affecting children with PCD are Haemophilus influenza and Staphylococcus aureus, with P. aeruginosa being more common in adults. Some subjects cultured more than one organism in the same sample: for example, smooth P. aeruginosa with another organism, most commonly nontuberculous mycobacteria [1].Although pneumonia is frequently seen, lung abscess is rare in KS [2]. Only two previous cases have been reported in medical literature. Of them, one abscess was caused by Streptococcus pneumoniae while the other was secondary to P. aeruginosa. In our patient, the lung abscess involved multiple organisms, namely, P. aeruginosa, MAC and Nocardia spp. To the best of our knowledge, this is the first such case report in English language literature. Nontuberculous mycobacterial infection is considered pathogenic and requires an aggressive multi-drug regimen for eradication. Pulmonary nocardiosis is typically regarded as an opportunistic infection and occurs mostly in immune compromised hosts or patients with underlying lung disease. However, approximately 50% of patients with nocardiosis do not have any predisposing disease. The isolation of Nocardia spp. from the respiratory tract of KS patients in itself, is an unusual finding. The clinical features of these cases are summarized in the table.

CONCLUSION:  Lung abscess infrequently complicates KS. A case of lung abscess with P. aeruginosa, MAC and Nocardia spp. in a patient with KS is presented here. Evaluation of sputum and BAL is often needed to identify organisms. Prompt and appropriate treatment of respiratory infections can minimize irreversible damage in such cases.

DISCLOSURE:  M Ali Javed, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):15S-e-16S. doi:10.1378/chest.136.4_MeetingAbstracts.15S-e
FREE TO VIEW

INTRODUCTION:  A major cause of morbidity seen in spinal cord injury are related to respiratory complications. Typically, thoracic spinal cord lesions do not cause respiratory compromise related to ventilatory muscle compromise. However, because the diaphragm is the major muscle of ventilation and inspiration (C3-C5 nerve roots), chronic respiratory failure may develop. We present the case of a patient whose initial spinal lesion caused by a gun-shot wound would not have contributed to persistent hypercapnia, however he developed a syringomyelia extending into the cervical area which led to chronic ventilatory insufficiency.

CASE PRESENTATION:  A 49 year old male with history of parapalegia secondary to gunshot wound to the back suffered in 1997 with spinal cord injury at the level of T5, was admitted to the medical intensive care unit for urosepsis and concomitant acute hypercapnic hypoxic respiratory failure. He required mechanical ventilation for the treatment of respiratory distress and was subsequently found to have Pseudomonal infection in the urine and methicillin resistant staphylococcus aureus bacteremia. He responded well to antibiotics and defervesced after approximately 24 hours, but the patient experienced hypercapnia during attempted ventilator weaning. Review of laboratory testing prior to his infectious complications revealed a persistently elevated serum bicarbonate (range 30–36) over the past month. He was successfully extubated to BiPAP on day 4 of his ICU course and was ultimately transitioned to BiPAP at night.An MRI of the spine was done (see Panel A) after extubation to assess the cause for his chronic respiratory failure and hypoventilation as a spinal lesion at the T5 level should not result in persistent hypercapnia. He was found to have a syrinx that had extended from the level of C4 –T11 of the spine.

DISCUSSIONS:  Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord. 50% of cases are due to congenital defects that become more apparent as the person develops (usually into adolescence or young adulthood), the vast majority of which are caused by Arnold-Chiari malformations. The other 50% are due to causes such as spinal cord tumor or hemorrhage, scarring due to previous spinal trauma, or kyphoscoliosis. Progressive post-traumatic cystic syringomyelia is an uncommon but a potentially clinically serious complication of spinal cord injury. Post-traumatic delayed development of syringomyelia, once thought to occur in only 1–4% of cases, is now increasingly recognized as a major cause of morbidity. Progressive signs and symptoms can develop as early as three months after spinal cord trauma or can occur as late sequelae as late as 32 years after spinal cord injury.It is likely that our patient developed chronic hypoventilation from the extension of the syrinx to the level of C4 which can cause weakness in both the diaphragm and other muscles of respiration. As was noted in the case described, the risk of chronic ventilator dependence is greatest among patients with injuries close to the C3 level and in patients older than 50 with underlying lung disease. Some patients with injuries at this level may, with aging, insidiously develop chronic hypoventilation which requires chronic ventilatory support decades after spinal cord injury.Though the patient seemed to do well with improvement on BiPAP at night, he may ultimately require continuous mechanical ventilation.

CONCLUSION:  In the case just described, as the original lesion at T4 should not have affected the patient’s ventilatory status further investigations for this relatively uncommon complication of spinal cord injury was warranted. The possibility of syringomyelia should be considered in any patient who develops chronic hypercapnia following a spinal cord injury as extension of a syrinx can lead to progressive respiratory failure.

DISCLOSURE:  Hiren Patel, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):16S. doi:10.1378/chest.136.4_MeetingAbstracts.16S-c
FREE TO VIEW

INTRODUCTION:  In this case, we present a 74 year old African American female with primary pleomorphic rhabdomyosarcoma of lung. This case is being reported because of the extreme rarity of primary pulmonary pleomophic rhabdomyosarcoma, particularly after 70 years of age.

CASE PRESENTATION:  The patient was a 74 year old female who presented to the Emergency Department with 3 weeks of progressive dyspnea on exertion, cough productive of blood tinged sputum, nausea and loss of appetite with a 20 pound weight loss, and fatigue. She has a 60 pack year smoking history and quit smoking 4 years ago. Her vital signs included an oral temperature of 97.9F, a blood pressure of 168/83 mm Hg, a heart rate of 110 beats per minute, and a respiratory rate of 18 breaths per minute. Her oxygen saturation was 98% while breathing oxygen at a flow rate of 2L/minute by nasal cannula. Physical examination revealed diminished breath sounds in the left lung field but was otherwise unremarkable. Chest X-ray demonstrated a pleural-based left lung mass and mediastenal widening and pleural effusion. CT scan of chest revealed a large soft tissue mass in the mediastinum and hilum as well as multiple subcentimeter nodules in the left upper lobe. Fiberoptic bronchoscopy revealed extrinsic compression of the left main stem bronchus by the mass. Transbronchial biopsies of the mass were obtained and the pleural effusion was sampled by thoracentesis. Biopsy and effusion results demonstrated large atypical cells with nuclear positivity for myf-4 (myogenin) and desmin, a pattern consistent with pleomorphic rhabdomyosarcoma. Stains for TTF-1, WT-1, CD-20, CD-30, LCA, EMA and AE1/AE3 were negative. Cancer was staged as Stage111b, after ruling out metastasis.Surgical treatment was avoided because of her poor medical status. The patient was treated with chemo-radiation. Unfortunately patient didn’t do well with treatment and expired after receiving two cycles of Chemotherapy.

DISCUSSIONS:  Rhabdomyosarcoma (RMSC) is a rare malignant tumor of rhabdomyoblastic origin. Histologically, it may take three forms: embryonal, alveolar, and pleomorphic. The embryonal variety is most frequently encountered (75% of all RMSC) and is typically seen in those younger than 15 years of age, The alveolar type is the second most common (20% of all RMSC) and the pleomorphic RMSC is exceedingly rare. Males are affected more than females and it is rare to diagnose this disease after the age of 70. All RMSC are highly malignant tumors. The cells closely resemble those found in small cell carcinoma of the lung and can be mistaken easily. Differentiation can be made using myogenin stains which are positive in RMSC. Surgery is the best treatment but chemo-radiotherapy is an option for nonsurgical candidates.

CONCLUSION:  Primary pleomorphic rhabdomyosarcoma is a rare malignancy of the lung and is extremely rare after the age of 70. It can appear histologically similar to small cell carcinoma of the lung but can be differentiated using myogenin stains. The appropriate diagnosis of PRMS is significant as it is a high-grade sarcoma, with an aggressive clinical course and grave prognosis.

DISCLOSURE:  Sudheer Nambiar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):16S. doi:10.1378/chest.136.4_MeetingAbstracts.16S-d
FREE TO VIEW

INTRODUCTION:  Primary lung angiosarcoma is a rare entity with poor prognosis. We present a patient with pulmonary epithelioid angiosarcoma misdiagnosed as granulomatous lung disease on pathology and try to iterate the importance of following expected symptom resolution with appropriate therapy.

CASE PRESENTATION:  36 year old non smoking man presented to the emergency room with progressively increasing hemoptysis over a 4 week period. He was coughing about 150 to 200 ml of fresh blood a day. No history of hematuria, weight loss, rheumatologic or vasculitic symptoms. CT chest revealed a 2.5 × 2.0 cm left hilar mass. CT guided fine needle aspiration and core biopsy of this mass was consistent with vasculitides, most probably wegener’s granulomatosis. Pulse steroids were administered with methyl prednisolone at a dose of 1 gram a day after confirming no fungal elements in the mass. One week into steroid therapy patient did not have a significant symptomatic or radiologic improvement. ANCA (anti-neutrophil cytoplasmic antibody) screen was negative. This prompted us to rebiopsy the mass under CT guidance. Fine needle biopsy revealed an invasive malignancy with tumor cells staining positive with CD31, cytokeratin AE1/3 and factor 8 consistent with epithelioid angiosarcoma. Both the initial and later pathology specimens were reconfirmed by a pulmonary pathologist. Subsequent staging revealed the tumor was confined to thorax suggesting primary pulmonary epithelioid angiosarcoma.

DISCUSSIONS:  Hemoptysis in a young patient with lung mass is concerning for primary pulmonary neoplasm versus non malignant etilogies like infection or inflammatory vasculitis. 5% of patients can have ANCA negative wegener’s granulomatosis. Although a combination of cyclophosphomide and steroids for 6 months could be necessary for complete remission of wegener’s, majorityof the patients with pulmonary vasculitis as the cause of hemoptysis respond to high doses of steroids alone. Angiosarcoma constitutes less than 1% of all sarcomas. The sites most frequently involved are the skin and subcutaneous tissue, liver, breast, and heart. Angiosarcoma occurring in the lung usually represents metastasis from the heart, the pulmonary arterial trunk, or an extrathoracic organ. Angiosarcoma of pulmonary origin is very rare, being reported only in a handful of cases. It usually presents with intractable hemoptysis or massive intraparenchymal or intrapleural hemorrhage. Differential diagnosis between pulmonary angiosarcomas and other masses, especially lung carcinoma and a variety of vascular lesions with epithelioid endothelial cells, is not possible without biopsy. The distinction between benign and malignant vascular lesions can be quite challenging, even on histopathological studies, and requires immunohistochemical analysis in most patients. The epithelioid form of angiosarcoma has been fully characterized only in the past decade. Among the various immunohistochemical markers used for classification, factor VIII-related antigen and CD31 are considered specific for tumors derived from the endothelium. The prognosis is poor with mortality approaching 100% within months of initial presentation. Therapeutic modalities such as radiation therapy, chemotherapy, and surgical intervention have all been attempted, but none of them have been shown to be dramatically effective.

CONCLUSION:  We present this case in attempt to bring forward the importance of expected results of any therapy and clinical followup which could be the only clues when pathologic diagnosis is in question and not agreeing with our clinical diagnosis. A second look at the tissue in such circumstances may steer us in the right direction.

DISCLOSURE:  Akhil Vallabhaneni, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: hemangiosarcoma , lung
Chest. 2009;136(4_MeetingAbstracts):16S-e-17S. doi:10.1378/chest.136.4_MeetingAbstracts.16S-e
FREE TO VIEW

INTRODUCTION:  Pulmonary artery sarcoma is a rare tumor of the cardiovascular system, first described by Mandelstramm in 1923. Here, we present a case of metastatic pulmonary artery intimal sarcoma mimicking saddle pulmonary embolism.

CASE PRESENTATION:  A 38-year-old male presented to his PCP with a six week history of progressive dyspnea on exertion, decreased exercise tolerance, frequent palpitations, and pleuritic chest pain. Chest radiography revealed two masses in the left upper lung. Chest CT confirmed a large mass in the left upper lobe, and smaller mass in the lingula. PET scan revealed uptake in the mediastinum. The patient underwent fine needle aspiration of the left upper lobe lesion, revealing low grade spindle cell tumor. Subsequently, diagnostic mediastinoscopy with lymph node dissection was performed. Pathology was negative for malignant cells. Five days later, the patient reported persistent dyspnea and pleuritic chest pain while in his pulmonologist’s office. Vitals revealed a significant tachycardia with a heart rate of 120. An emergent CT Angiogram of the chest revealed bilateral pulmonary emboli and an apparent saddle embolism within the main pulmonary artery. He was hospitalized and initiated on anticoagulation therapy with Heparin, and an IVC filter was placed. Despite a presumed large clot burden, the patient remained hemodynamically stable, and transthoracic echocardiogram revealed normal right and left heart function with normal pulmonary artery pressures. His symptoms improved and he was discharged home on Lovenox. One week later, he again developed worsening dyspnea and recurrent chest pain. Repeat CT angiogram of the chest did not reveal any significant changes. A VQ Scan was then obtained, showing multiple segmental and subsegmental abnormalities consistent with pulmonary emboli. The greatest perfusion defects were noted in the lingula, right middle lobe and right lower lobe. Given his lack of response to anticoagulation therapy, the diagnosis of possible pulmonary artery sarcoma was entertained. He was transferred to a facility specializing in pulmonary endarterectomy. An MRI of the chest confirmed suspicion of a mass in the pulmonary artery. He eventually underwent right and left pulmonary endarterectomy. Pathology revealed thrombus as well as intimal sarcoma. He is currently undergoing chemotherapy with Sunitinib 50mg daily, which he is tolerating well.

DISCUSSIONS:  Pulmonary artery sarcomas usually arise from the dorsal pulmonary trunk, growing along the intima of the arterial wall in the direction of blood flow. They can also arise from the right and left pulmonary arteries, the pulmonary valve, and the right ventricular outflow tract. Histology most often reveals angiosarcoma. Our patient’s histology showed intimal sarcoma, which is rare and distinct from angiosarcomas in that it is highly cellular, radiographically dense, and poorly responsive to chemotherapy.Review of the literature reveals five case reports of pulmonary artery sarcoma initially presenting as pulmonary embolism. Common symptoms include hemoptysis, dyspnea (75%), and chest pain or cough (50%). Patients may also develop weight loss, fever, anemia, and digital clubbing. Other subtle findings may include an elevated erythrocyte sedimentation rate, the absence of a procoagulant state, and lack of prior deep vein thrombosis history. Historically, mean survival is 18 months, and 5-year survival is 18.5%. Surgical resection with curative intent portends the best survival. Multi-modality therapy, involving surgical resection with neoadjuvent chemotherapy, is currently recommended.

CONCLUSION:  Pulmonary artery intimal sarcoma is a rare tumor which carries a poor prognosis. Diagnosis is often delayed, as symptoms are often subtle and easily mistaken for thromboembolic disease. Failure to appropriately respond to anticoagulation should prompt consideration of this diagnosis.

DISCLOSURE:  Alison Kole, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):17S. doi:10.1378/chest.136.4_MeetingAbstracts.17S-d
FREE TO VIEW

INTRODUCTION:  Pulmonary alveolar microlithiasis (PAM) is a rare disease of unclear etiology characterized by diffuse formation of microscopic calculi or microliths within the alveoli. We present a unique case report of a patient with a rare type of immunodeficiency who developed alveolar microlithiasis.

CASE PRESENTATION:  A two year old male was referred to our clinic for assessment of chronic lung disease. He presented with chronic cough and intermittent tachypnea with minimal response to inhaled steroids and beta agonists. He had an undefined immunodeficiency consisting of low B cells, low natural killer cells and agammaglobulinemia requiring weekly immunoglobulin infusions. Previous respiratory illnesses included prolonged intensive care admission with Adult Respiratory Distress Syndrome, as well as treatment for presumed pneumocystis jiroveci. Noninvasive home ventilation had been required for respiratory insufficiency in the past. Chest radiograph revealed a diffuse pattern of interstitial lung disease. Imaging by CT scan demonstrated ground glass opacities with mild bronchial wall thickening. On subsequent transbronchial biopsy, areas of intraalveolar calcification were noted with minimal fibrotic thickening of septae. An open lung biopsy was performed which revealed bulky mineralization scattered throughout a fibrotic, chronically inflamed parenchyma. Areas of calcification were again localized to the alveoli. Serum levels of calcium, phosphate and parathyroid hormone were within normal limits.His course was further complicated by development of pancytopenia which led to a diagnosis of monosomy 7 and myelodysplastic syndrome. It was decided to proceed to bone marrow transplant due to rising peripheral blast percentage and further pneumocysitis jiroveci infection. CT chest post transplant demonstrated more confluent groundglass opacities with calcified centrilobular nodules and scattered bronchiectasis. Pulmonary symptoms remained stable.

DISCUSSIONS:  The etiology of PAM is unknown but familial occurrence is documented in many cases with an autosomal recessive pattern of inheritance. PAM has been noted in patients with other disorders such as azoospermia, lymphocytic interstitial pneumonitis, mitral stenosis and milk alkali syndrome. There have been no previously documented cases of patients with PAM and either immunodeficiencies or hematological disorders requiring bone marrow transplant. The impact of bone marrow transplant on PAM is unknown, however in this case to date pulmonary symptoms remain stable although radiological findings show progression of pulmonary disease.Many patients with PAM are asymptomatic and the presence of respiratory symptoms depends on the extent of pulmonaryalveolar calcification. When the disease is extensive respiratoryfailure can lead to cor pulmonale and lung transplantation with variable results.The diagnosis of PAM can be suspected on chest radiograph if the typical “sandstorm” appearance is noted. This was not present in our case and further evaluation by CT chest was used to demonstrate significant ground glass opacities. Most pediatric cases of PAM are confirmed on transbronchial biopsy where, as in this case, lung parenchyma demonstrates microliths in the alveolar spaces with associated thickened fibrotic interstitium. Pathogenesis of PAM appears to be related to deposition of calcium phosphate salts in the alveoli either by extrusion from pulmonary capillaries or other unknown mechanisms.No specific therapy has been reported for PAM and lung transplantation has been the only option for those with progressive disease. Varied results have been found with use of glucocorticoids, calcium and phosphate binders and surfactant use. No specific therapy has been advocated in our patient to date, however the use of immunosuppressant therapy may be playing an unclear role in symptom prevention.

CONCLUSION:  PAM is a rare disease of unclear etiology and this case demonstrates the importance of histological diagnosis in complex cases. We also document the first case associated with hematological malignancy and disease progression post bone marrow transplant.

DISCLOSURE:  Fiona Healy, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):17S-e-18S. doi:10.1378/chest.136.4_MeetingAbstracts.17S-e
FREE TO VIEW

INTRODUCTION:  Peanut allergy is one of the leading food-related allergies with significant morbidity and mortality. Immunoglobulin E (IgE)-mediated type 1 hypersensitivity to peanut is a leading cause of anaphylaxis. Anaphylaxis is a mast cell dependent process mediated by IgE. Mast cells sensitized by peanut-specific IgE can initiate the anaphylaxis cascade resulting in multisystem involvement, including cardiovas cular, respiratory, gastrointestinal, neurologic, and dermatological manifestations. Transfer of IgE-mediated hypersensitivity to peanuts following transplantation is rare and has only once been previously described following lung transplant.(1) The mechanism for allergy transfer following transplantation is postulated to result from transfer of IgE-producing donor B cells or from passive transfer of sensitized mast cells. While serum IgE has a short half-life of days, mast cell bound IgE may persist for up to 6 months and cause allergic symptoms or anaphylaxis upon allergen exposure.

CASE PRESENTATION:  A 47-year-old female underwent bilateral lung transplantation for non-specific interstitial pneumonitis (NSIP) and received donor lungs from a twelve-year-old patient with a known allergy to peanuts. The recipient was not known to have a history of tree nut or peanut allergy, but had a history of generalized urticaria following ingestion of shellfish. Post-transplant, the patient experienced four anaphylaxis-like reactions (flushing, itching, hypotension) after consuming cereal, coconut cream pie (on two occasions), and chocolate. No single episode involved clear peanut or tree nut consumption; however, consumed products were not certified as peanut free. Searching for a causal agent, initial skin prick test (SPT) to coconut and subsequent oral coconut challenge were negative. Skin prick tests to tree nuts were negative; however, peanut SPT was 4+ positive (8×7mm). Interestingly, radioallergosorbent test (RAST) for peanut-specific IgE was negative at <0.35kU/L. The patient was counseled to avoid peanut exposure and carry an epinephrine auto-injector (EpiPen®). The positive result of peanut SPT testing steadily declined over serial assessments and reverted to negative one year post-transplant. The patient declined oral peanut challenge following the negative peanut SPT.

DISCUSSIONS:  Transfer of food allergy post-transplantation is theorized to occur via transfer of donor B cells producing peanut-specific IgE into the circulation of the recipient. An alternate mechanism proposes passive transfer of IgE-sensitized mast cells within the transplanted tissue that then migrate into recipient tissues. A negative RAST assay in our patient suggests the absence of ongoing peanut-specific IgE production. Positive peanut-specific SPT supports the tissue presence of mast cells sensitized by peanut-specific IgE. The gradual decline in the magnitude of the peanut SPT and its return to negative over the course of one year supports the gradual depletion of donor sensitized mast cells in recipient tissue and supports the passive transfer of sensitized mast cells from donor tissue during transplantation.

CONCLUSION:  Passive transfer of allergen-sensitized mast cells following organ transplantation can result in donor anaphylaxis and death. Lack of an anaphylaxis history in a donor may not exclude the possibility of the recipient experiencing an anaphylactic episode. However, detailed donor allergy histories and recipient avoidance of known donor-sensitive allergens can reduce the morbidity and mortality associated with post-transplantation anaphylaxis. Post-transplantation skin prick testing of recipients may be appropriate in the setting of donors with severe allergy to predict those at greater risk of anaphylactic episodes.

DISCLOSURE:  Sacha Bhinder, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):18S. doi:10.1378/chest.08-2354
FREE TO VIEW

INTRODUCTION:  Since 2001, increasing attention has been drawn to immunoglobulin G4 (IgG4) associated lymphoplasmacytoid infiltration, inflammation and fibrosis. This rare hyperIgG4 disease of unknown etiology has been increasingly recognized in pancreas, salivary glands, liver, lachrymal glands, kidney, aorta as well as lung. We report a case of atypical IgG4 positive lymphoplasmacytic cell infiltrate of the lungs; that had the disease process for 10 years before diagnosis.

CASE PRESENTATION:  A 43-year old African American male with history of right eye optic neuritis, migraine, beta thalassemia trait presented to our Institute with chronic dry cough, generalized body ache and fatigue for several months. His past history was significant for extensive work up of bilateral upper lobe lung infiltrates and fever 10 years ago. He had an open lung biopsy which revealed atypical lymphoplasmacytic cells infiltrating the lung. On flow cytometry no monoclonality demonstrated. No conclusive diagnosis was reached; the lung lesions underwent a spontaneous resolution. Almost five years before presentation he was diagnosed with inflammatory bowel disease which responded to a short course of mesalamine. The patient had quit smoking 10 years ago and before that he used to smoke 1 pack per day. He worked as a tailor. His family, social and personal history were non contributory. On examination the patient’s vitals were with in normal range, chest exam was significant for wheezing and inspiratory crackles in left interscapular region. Chest roentgenogram revealed a nodular lesion in the left lower lobe apical region. The pulmonary function tests showed restriction with reduced diffusion for carbon monoxide. Computerized tomogram revealed a 2.7 cm soft tissue mass involving the left lower lobe apical segment. Transbroncial biopsies on fiberoptic bronchoscopy revealed a lymphoplasmacytoid infiltrate similar to the one seen 10 years ago. Connective tissues diseases and HIV work up were negative. He had elevated ESR and CRP level. His work up for human herpes virus type 6 DNA, parvovirus B19 DNA was negative. PET scan showed abnormal FDG uptake in superior segment of left lower lobe with SUV of 4.7. Patient underwent repeat computerized tomography guided needle biopsy of left lung mass, which showed atypical lymphoplasmacytic infiltrate. A scan of the abdomen revealed left hydronephrosis with hydroureter without obstruction. A repeat computerized tomography guided needle biopsy was consistent with hyperIgG4 disease of the lung. Patient was initiated on 60 mg of prednisone per day with gratifying clinical as well as radiological improvement.

DISCUSSIONS:  HyperIgG4 disease is characterized by chronic inflammatory state. HyperIgG4-related lung disease can be categorized into four types on the basis of the predominant CT findings: solid nodular, round-shaped ground-glass opacity, alveolar interstitial, and bronchovascular. Our patient 10 years ago had interstitial disease process which spontaneously resolved, and at current presentation had a nodular disease process. The nodular disease process responded to therapy with prednisone. We believe the inflammatory bowel disease our patient had 5 years ago, hydroureter and hydronephrosis was also a part of the hyperIgG4 disease clinical manifestation.

CONCLUSION:  Our case highlights the need to increase the awareness of the disease process in the clinicians as well as pathologists. The delay in diagnosis can lead to increased morbidity and unneccessary invasive procedures as was seen in our case. For a decade he underwent repeated bronchoscopies and biopsies by interventional readiology. The multisystem involvement further complicates the clinical picture.

DISCLOSURE:  Ketan Patel, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):18S-d-19S. doi:10.1378/chest.136.4_MeetingAbstracts.18S-d
FREE TO VIEW

INTRODUCTION:  Pulmonary langerhans cell histiocytosis (PLCH) is a rare smoking-related interstitial lung disease. Pulmonary hypertension (PH) is a common complication, and is strongly associated with increased mortality. Our patient developed severe PH secondary to an unusual combination of pulmonary arteriopathy and pulmonary veno-occlusive disease (PVOD) associated with PLCH. The PH and the dyspnea improved with Bosentan therapy.

CASE PRESENTATION:  A 51 year old male with 60 pack year smoking history, presented with one year history of progressive exertional dyspnea, intermittent dry cough, and declining functional capacity. Physical examination was remarkable for decreased breath sounds bilaterally with diffuse end inspiratory fine crackles. Oxygen saturation was 89% and the PaO2 was 55 mm Hg on room air. Chest radiograph showed diffuse nodular interstitial opacities which were confirmed by high-resolution computed tomography that showed reticulonodular pattern with upper zone predominance. Pulmonary function testing revealed a mild combined obstructive and restrictive defect with a severe impairment in diffusing capacity at 40% of predicted suggesting pulmonary vascular disease. This was confirmed by an echocardiogram where the systolic pulmonary artery pressure was estimated at 98 mm Hg. Pulmonary embolism was not detected by perfusion lung scan or pulmonary angiogram. Hematologic, biochemical, and serologic tests were within the normal range. A right heart catheterization confirmed severe PH and there was no evidence of an intracardiac left-to-right shunt. Video assisted thoracoscopy with a wedge lung biopsy was performed which confirmed the diagnosis of PLCH with extensive interstitial fibrosis, medial hypertrophy and vascular intimal fibrosis with luminal obliteration consistent with PH and PVOD, respectively. The patient stopped smoking, treatment was started with prednisone and non-selective endothelin-1 blocker (Bosentan), and then the patient was referred to a lung transplantation center. Within 4 weeks, dyspnea on exertion improved. Pulmonary function tests and blood gases remained unchanged over 3 months. Right heart catheterization was repeated after 3 months of Bosentan therapy and demonstrated an important reduction of pulmonary artery pressure and pulmonary vascular resistance.

DISCUSSIONS:  In the literature, pulmonary arteriopathy and PVOD have been described as possible causes of PH in PLCH. Our patient had severe PH out of proportion to ventilatory limitation, secondary to both pulmonary arteriopathy and PVOD. The normal pulmonary artery occlusion pressure (PAOP) in our patient could be explained by the exit of tributaries from the occluded vein proximal to the stenosis and their connection with non-occluded veins eventually reaching the left atrium. Treatment for PH in PLCH is not well established. A better understanding of the pathogenesis in PLCH is needed to appropriately treat the underlying PH. Variable degrees of fibrosis and distortion of the normal pulmonary architecture are often associated with advanced PLCH, with some histologic overlapping with idiopathic pulmonary fibrosis (IPF). Endothelin-1 may underlie the pathogenesis of lung fibrosis. Bosentan (non-selective endothelin-1 blocker) may have efficacy for the treatment of PH in advanced fibrotic phase of PLCH. A recent randomized control-blinded trial (BUILD-1) showed that Bosentan improved the quality of life and delayed time to death in IPF. However, pulmonary edema occurred in some patients with PVOD who were treated with oral Bosentan. Pulmonary arteriopathy contributes to the development of PH in PLCH. Prostacyclin induces relaxation of vascular smooth muscle by stimulating the production of cAMP and inhibits the growth of smooth-muscle cells. However, acute pulmonary edema occurred in some patients with PLCH who were treated with intravenous prostacyclin, due to the concomitant PVOD. This effect probably resulted from the increase in pulmonary blood flow in the setting of downstream vascular obstruction.

CONCLUSION:  PLCH is often associated with severe PH and many new options for treatment are currently available but multicenter clinical trials are needed to develop an evidence-based approach.

DISCLOSURE:  Bassel Ericsoussi, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):19S. doi:10.1378/chest.136.4_MeetingAbstracts.19S-d
FREE TO VIEW

INTRODUCTION:  Pyoderma Gangrenosum (PG) is a rare inflammatory disorder of the skin characterized by the presence of irregular ulcers with violaceous borders, often in the setting of systemic disease. The histopathology features non-specific neutrophilic infiltration; the pathogenesis is thought to involve dysfunction of neutrophil chemotaxis. Extracutaneous manifestations are rare but pulmonary involvement has been reported. We report a case of a pulmonary PG and acute respiratory distress.

CASE PRESENTATION:  This 61-year-old never-smoker was admitted with hypoxic respiratory failure. He was in good health until 2 years ago when he suffered multiple non-healing ulcers on his arms and abdomen originally diagnosed as necrotizing fasciitis. He failed multiple therapies and had consistently sterile cultures. Biopsy showed neutrophillic infiltrates. He was diagnosed with PG and treated with infliximab resulting in prompt resolution of symptoms. Initial evaluation for concurrent systemic disease revealed splenomegaly, for which he underwent a splenectomy. Subsequently he developed thrombocytosis and monocytosis, and a bone marrow biopsy confirmed the diagnosis of underlying CMML for which he was treated with hydroxyurea. Six weeks prior to admission he redeveloped suspicious skin lesions and was restarted on infliximab. He then presented to the emergency room with a one-week history of progressive dyspnea, weakness and fatigue. Physical examination revealed a well-nourished male in acute respiratory distress, fever of 101.7, tachypnea and accessory muscle use. Coarse crackles were auscultated bilaterally on chest exam. Laboratory examination showed a WBC of 9500 with 3% bands, 22%. Platelet count was 355,000. ABG showed a paO2 of 67 mmHg on 40% FiO2. Chest x-ray showed bilateral pulmonary infiltrates. A CT angiogram revealed centrilobular and peribronchial ground glass infiltrates. An echocardiogram showed a normal ejection fraction and moderate pulmonary hypertension. He was immediately started on broad spectrum antibiotics. His respiratory status continued to decline and he required intubation and mechanical ventilation. Interestingly, he had rapid improvement of symptoms initially and was extubated within 2 days only to be re-intubated within 48 hours. Over the following 6 weeks, the patient fell into a pattern of improved respiratory status and extubation followed by recurrence of hypoxic respiratory distress and re-intubation. He had in the interim been started on empiric corticosteroid therapy and antifungal therapy as well as given a tracheostomy. Multiple bronchoalveolar lavages failed to reveal an infectious or malignant source of his symptoms. Repeated cultures were negative. Serological studies and inflammatory markers were nondiagnostic. The patient then underwent a surgical lung biopsy. Pathology showed a uniform process of numerous fibroblastic foci, thickening of alveolar septi, and focal areas of acute inflammation without granulomas. It was then decided to treat the patient empirically for PG involvement of the lungs with 1g methylprednisolone daily for three days. The patient’s respiratory status dramatically improved allowing liberation from mechanical ventilation for the rest of his hospitalization. Review of the patients chart demonstrated a correlation between steroid tapers and decline of respiratory function.

DISCUSSIONS:  Our patient’s profound response to corticosteroids led us to believe that he had PG involvement of his lungs. We suspect this particularly because he had a recent flare of cutaneous PG. Our pathologic findings are consistent with PG although non-diagnostic. Other possibilities do exist, including drug-induced pneumonitis secondary to either hydroxyurea or infliximab.

CONCLUSION:  While pulmonary involvement of PG is rare it should be considered in a patient with PG and respiratory symptoms.

DISCLOSURE:  Michael English, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):19S-e-20S. doi:10.1378/chest.136.4_MeetingAbstracts.19S-e
FREE TO VIEW

INTRODUCTION:  Diffuse pulmonary ossification (DPO) is a rare disease characterized by diffuse small bone fragments in the lung tissue. It is usually discovered at autopsy. We describe a case where DPO was detected on lung biopsy for evaluation of Interstital lung disease (ILD).

CASE PRESENTATION:  A 72 yr-old man was admitted for midsternal chest pain and shortness of breath. He reported gradual worsening of dyspnea and cough over a few years. Other medical history included diabetes, Hypertension and chronic renal insufficiency. He was an Ex-smoker with no significant environmental or recreational risk exposure. Physical examination revealed bilateral basilar ronchi. Chest radiograph revealed fine nodular interstitial pattern. A high resolution chest CT revealed extensive peripheral pulmonary fibrosis, “bright” nodularity and interlobular septal thickening (Fig 1). Video assisted thoracoscopic lung biopsy revealed patchy interstitial fibrosis with metaplastic bone formation generally in association with fibrotic foci with a background of pan-acinar emphysema (Fig 2). These findings were consistent with DPO of the “nodular” variety.

DISCUSSIONS:  Pulmonary ossification is defined by the histologic presence in the lung of mature bone often containing marrow elements. This can be either a primary idiopathic process or secondary to a number of pulmonary, cardiac, orextracardiopulmonary disorders. Diffuse Pulmonary Ossification (DPO), may be either granular or dendriform. The nodular form is characterized by lamellar deposits of calcified osteoid material situated within the alveolar spaces often without marrow elements. Dendriform ossification refers to interstitial branching spicules of bone and marrow elements that may protrude into the alveoli and often follows interstitial fibrosis.Tissue injury is the most important provoking factor. An alkaline environment, initiates precipitation of calcium salts, enables alkaline phosphatase activity, and activates profibrogenic cytokines. Alveolar bleeding is responsible for interstitial metallic deposition that attracts calcium salts and multinucleated giant cells..DPO is most commonly found in men between 70 and 80 years of age, but cases have been reported in young men and women. Many cases are diagnosed at autopsy. The signs, symptoms, and physiological abnormalities pointing to DPO may be secondary to another process. Pulmonary ossification is not usually visible in chest x-rays. When visible, it appears in the inferior lobes as an unspecified reticulonodular density. In most cases it is difficult to determine whether the very fine lines in the x-ray image indicate calcified areas or pulmonary fibrosis. Consequently, the disease is usually discovered by chance during autopsy. High resolution CT scans of the thorax reveal lines of 1 to 4 mm in the form of either branching calcifications of bronchovascular distribution in the dendriform type or multiple areas of tiny calcified subpleural nodules in the nodular type. Establishing a prognosis is difficult as few living cases are diagnosed. Some case reports describe no changes of interest over time; others describe a slow evolution.The literature contains no cases of spontaneous regression.

CONCLUSION:  Diffuse pulmonary ossification is under-recognized and under-reported. Its pathological significance remains unknown. Creation of a registry and continued follow-up of these patients may pave the way for better understanding of pathogenesis and natural course of this disease.

DISCLOSURE:  Andres Escobar Naranjo, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: lung , osteogenesis
Chest. 2009;136(4_MeetingAbstracts):20S. doi:10.1378/chest.136.4_MeetingAbstracts.20S-c
FREE TO VIEW

INTRODUCTION:  Hughes-Stovin syndrome is a rare syndrome characterized by peripheral venous thrombosis, cerebral venous sinus thrombosis and recurrent pulmonary artery aneurysms (PAA). Aneurysms in the systemic circulation can also be seen. We report the first case of Hughes-Stovin syndrome with coronary artery aneurysm.

CASE PRESENTATION:  A 25 year old male presented to an outside hospital with a history of hemoptysis, fatigue and dyspnea for 4 months. Chest CT scan revealed a large right upper lobe PAA. Due to uncontrollable hemoptysis, patient had a right upper lobe lobectomy. Post-operatively he developed ventilator dependent respiratory failure and deep venous thrombosis (DVT) in all four extremities. Hemoptysis recurred, and repeat CT scan showed new PAA in right lower lobe and left lung. Patient was transferred to our institution. On arrival, he complained of intermittent hemoptysis, dyspnea and weakness. He denied joint pain, oral or genital ulcers, rash, or visual changes. He had a history of congenital hydrocephalus treated with a ventriculo-peritoneal shunt and dural sinus thrombosis requiring warfarin anticoagulation. He was a non-smoker and did not use illicit drugs. Physical exam and basic laboratory studies were normal.Rheumatologic workup was significant for weakly positive anti-histone antibodies and elevated C-reactive protein. Chest CT scan showed four PAA, the largest of which was partially thrombosed. There was a 2.2 cm vascular lesion in the posterior aspect of the left ventricle consistent with a coronary artery aneurysm. Based on combination of PAA, DVT, and sagittal sinus thrombosis Hughes-Stovin syndrome was diagnosed. He was started on intravenous methylprednisone 250mg Q6H for three days and received cyclophosphamide 750 mg loading dose. His hemoptysis decreased significantly within 72 hours He was discharged on oral cyclophosphamide and prednisone. At two weeks follow up he was asymptomatic.

DISCUSSIONS:  Hughes-Stovin syndrome is a rare disorder of unknown etiology first described in 1959. The authors described four males with DVT, cerebral venous sinus thrombosis and PAA. They hypothesized the PAA were due to degenerative changes in bronchial arteries or infected emboli from low virulence organisms. Hughes-Stovin syndrome has been postulated to be a variant of Behcet’s disease due to some similarities in clinical and histopathological findings. Systemic vasculitis has been suggested as etiology due to involvement of systemic circulation. It commonly occurs in young men. Clinical features include recurrent fever, chills, cough and hemoptysis that can be massive and fatal. Other features include both superficial and deep venous thrombosis, signs of elevated intracranial pressure and pulmonary hypertension. Aneurysms in systemic circulation have been reported in multiple case reports. Our patient is the first reported case of coronary aneurysm in Hughes-Stovin syndrome.Hughes-Stovin syndrome should be suspected in any young patient with hemoptysis and PAA in the absence of clinical features of Behcet’s disease. Chest CT scan is obtained to detect PAA.Given its similarities with Behcet’s diseases; treatment with corticosteroids and immunosuppressive agents has been suggested.

CONCLUSION:  1) Hughes-Stovin syndrome is a rare disorder characterized by PAA and venous thrombosis. 2) Systemic vasculitis has been suggested as etiology, and thus it can affect any systemic circulation. In our patient it caused a coronary artery aneurysm. 3) The combination of corticosteroids and Cyclophosphamide appears to be effective treatment for this rare and potentially fatal disorder.

DISCLOSURE:  Hiren Shingala, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):20S-d-21S. doi:10.1378/chest.136.4_MeetingAbstracts.20S-d
FREE TO VIEW

INTRODUCTION:  Hereditary Hemorrhagic Telengiectasia (HHT), an autosomal dominant (AD) disease, causes vascular malformations (telengiectasias), mainly on the skin, mucous membranes, lungs, liver and the brain. This leads to recurrent hemorrhages causing varying degrees of morbidity. The two main subtypes characterized by different genetic abnormalities are HHT-1 and HHT-2, defects of which are found on chromosome 9 (9q33–34) in the gene encoding Endoglin, and chromosome 12 (12q13) in the gene encoding Activin Receptor Like Kinase-1 (ACVRLI) respectively. Both gene products are members of the Transforming Growth Factor-Beta (TGF-×) receptor family, and are involved in regulation of angiogenesis. There is variable degree of penetrance leading to variations in disease phenotype.

CASE PRESENTATION:  A 33 years old man who presented with recurrent epistaxis and joint pains involving the wrist, fingers and ankles was diagnosed initially with Rheumatoid Arthritis (RA) and started on therapy with Prednisone and Hydroxychloroquine. His symptoms quickly resolved except for the epistaxis. A year later, while still on Prednisone, he developed worsening shortness of breath with a declining functional status over a few months. Computed tomography (CT) of the chest revealed extensive areas of abnormality predominantly located in the posterior segments of the lower lobes bilaterally, described as interstitial thickening, honeycombing with traction bronchiectasis suggestive of alveolar fibrosis. A right lower lobe AVM was also seen which was coiled successfully. 6 years later during a workup for an acute decline in respiratory status another AVM was found and coiled in the left upper lobe, while his parenchymal disease remained unchanged. He was continued on Prednisone throughout this time. Genetic testing revealed an Endoglin gene abnormality (HHT-1). Parental testing did not show any genetic abnormality. Attempts at reducing the dose of Prednisone was unsuccessful, resulting in worsening respiratory status, new areas of ground glass opacities on CT imaging and increased oxygen requirement at rest. Spirometry over an 8 year period showed a steady decline with severe restrictive defect although lung parenchyma on CT scan remains stable. During this time, while maintained on Prednisone patient has not had any other organ involvement. He does not have any skin or mucus membrane abnormalities. Based on his current clinical condition requiring 4 liters/ minute nasal canula oxygen flow at rest and progressive decline in lung function patient has been referred for lung transplantation.

DISCUSSIONS:  There are several interesting aspects of this case. First, the coexistence of HHT and RA has never been reported in the literature. Unfortunately an open lung biopsy was never performed and the abnormalities seen on CT scan are not clearly defined. The initial insult could have resulted from either rheumatoid lung disease or bleeding from AVM’s. Worsening infiltrates while attempting a steroid taper favors the former. Second is the occurrence of HHT in our patient with genetically normal parents. Third is the presence of severe and progressive parenchymal lung disease as the only active manifestation of RA while on systemic therapy.

CONCLUSION:  HHT is an aggressive AD disease, which in spite of its mode of inheritance could lead to a skip generation, mainly due to reduced penetrance. The gene product of this mutation may be linked with other disease processes including connective tissue diseases and cancers.

DISCLOSURE:  Obaid Awan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):21S. doi:10.1378/chest.136.4_MeetingAbstracts.21S-d
FREE TO VIEW

INTRODUCTION:  Poland syndrome is a rare congenital abnormality of the chest wall that has been associated with many different malignancies. To our knowledge, we present the first case of Poland’s syndrome associated with extragonadal seminoma.

CASE PRESENTATION:  A 32 year-old Honduran man presented with a subacute history of recurrent hemoptysis associated with dull chest pain, dyspnea on exertion, dysphagia, weight loss and fevers. Past medical history was significant for congenital asymmetry of the chest and tobacco abuse. On examination the left anterior chest wall appeared asymmetrically sunken with diminished breath sounds over the LUL. Admission WBC was 16.1 K/uL and LDH 240 IU/L. CXR revealed a 7.5 cm LUL mass with an adjacent 2.8 cm thick walled cavity and hyperlucency of the remaining the left hemithorax. CT chest additionally showed absence of the left pectoralis major muscle, narrowing of both the left pulmonary artery and LUL bronchus, and mediastinal lymphadenopathy. Imaging studies for extrathoracic spread of disease and fungal serologies were negative. β-HCG, α-FP and CEA tumor markers were unremarkable. Sputum cytology was positive for cells suggestive of but not diagnostic for malignancy. Transbronchial and subcarinal biopsies showed a highly unusual tumor loosely resembling adenocarcinoma. Immunohistochemical staining favored a diagnosis of extragonadal seminoma. Chemotherapy was started but upon follow-up the disease had progressed significantly, manifested by further mass enlargement, mediastinal shift, tracheal compression, a pleural effusion, WBC of 41.3 K/uL and respiratory stridor. Urgent radiation therapy was started and a biopsy of a new left chest wall lymph node was obtained. Repeat immunochistochemical results were unchanged. Flow cytometry ruled out lymphoma. Microscopic evaluation revealed cells with large pleomorphic atypical nuclei and abundant clear cytoplasm mixed with numerous mature lymphocytes on a lacy linear background producing a “tigroid” pattern. Finally, chromosomal analysis showed the presence of multiple clonal abnormalities in all cells, including extra copies of 9q and of 12p. These findings were felt to be diagnostic of seminoma and second line chemotherapy was started. Ultimately there was no clinical response and the patient expired.

DISCUSSIONS:  Congenital abnormalities of the chest are rare in the adult population. When the abnormality results in partial depression of the anterior chest wall the differential diagnosis includes pectus excavatum, anterior thoracic hypoplasia, Poland’s syndrome, and skeletal dysplasia. Although radiographically distinguishable, only Poland’s syndrome has been associated with malignancy. Poland’s syndrome is characterized by the absence of at least the costosternal portion of the pectoralis major muscle combined with a wide spectrum of hypoplasia or aplasia of the ipsilateral breast, nipple, pectoralis minor muscle, ribs and hand. Although an environmental interruption of the embryonic limb blood supply is the favored etiologic theory, genetic defects cannot be ruled out given reports of several familial occurrences. Poland’s syndrome has been associated with a variety of malignancies including leukemia, non-Hodgkin’s lymphoma, leiomyosarcoma and breast cancer. Classic findings of seminoma, as in our case, include 12p amplification, lymphocytic infiltration, and a “tigroid” background. Previous reports on malignancy with Poland’s syndrome have not described an aggressive predilection of the associated cancer as was seen in this case.

CONCLUSION:  In adults presenting with a congenital depression of the chest wall and a lung mass, the likelihood of Poland’s syndrome is high. Similar to other congenital abnormalities, Poland’s syndrome is associated with malignancy but to date no genetic cause has been identified. The clinical course described emphasizes the importance of persistent aggressive tissue evaluation when a diagnosis remains uncertain. Adults with Poland’s syndrome require vigilant cancer screening.

DISCLOSURE:  David Sonetti, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):21S-e-22S. doi:10.1378/chest.136.4_MeetingAbstracts.21S-e
FREE TO VIEW

INTRODUCTION:  In 1951, Churg and Strauss characterized a form of necrotizing vasculitis, with eosinophilic tissue inflammation and extravascular granulomas occurring in individuals with asthma, allergic rhinitis or sinusal polyposis (Churg-Strauss Syndrome (CSS)). This rare disease has an annual incidence of 0.5- 6.8 per million persons. Like many other vasculitides, CSS has diverse clinical manifestations and is often diagnostically challenging.We report a case of CSS in a 43-year-old female patient without pre-existing asthma, allergic rhinitis or a personal atopic history who presented with generalized lymphadenopathy.

CASE PRESENTATION:  A previously healthy 43-year-old Caucasian female presented complaining of a one-month history of dry cough and progressive generalized lymphadenopathy of two-week duration. The patient reported concurrent fever, malaise, weight loss, and night sweats. Several days prior to admission, a pruritic maculopapular rash in the lower trunk had erupted causing much discomfort. Her medical history was negative for asthma, allergic rhinitis or atopy. No prescription medication and no known drug allergy were elicited on history. Her social history and family history were unremarkable. On physical examination she was febrile (39.2° C); her other vital signs were normal. Bulky (up to 2cm in diameter), and slightly tender rubbery mobile lymph nodes were noted bilaterally in the anterior cervical chain, Waldeyer’s ring, axilla, and inguinal region and her spleen was enlarged. An erythematous maculopapular rash was noted over the lower trunk, in addition to bilateral shin petechiae. The rest of her examination was normal. Initial laboratory investigations were notable for an elevated white blood cell count (15.1×109/L; reference range 4.0–11.0 ×109/L), eosinophil count (2.1×109/L; reference range 0.0–0.7×109/L), sedimentation rate (23mm/h; reference range 0–20mm/h), alkaline phosphatase (214U/L; reference range 30–115U/L) and alanine aminotransferase (164U/L; reference range 1–40U/L). Her initial chest radiograph was normal but within a few days she developed bilateral, patchy airspace disease that corresponded to hypoxemia. This patient underwent vasculitis serology, CT chest and abdomen, bronchoscopy, and excisional lymph node biopsy. CT showed bilateral patchy ground glass opacities, mediastinal and axillary lymphadenopathy as well as diffuse adenopathy in the abdomen. Histological sections of the lymph node showed the presence of granulomas containing central eosinophils and necrosis. Multiple needle-shaped Charcot-Leyden crystals were observed. In addition, the transbronchial biopsy findings of eosinophilic pneumonia with non-necrotizing vasculitis were also compatible with the final diagnosis. Subsequent blood work revealed an elevated immunoglobulin E level (196.2 KIU/L; reference range <160KIU/L), and a positive p-ANCA (Anti-MPO 9.7 KEU/L; reference range<5.0KEU/L). This patient responded initially to treatment with oral prednisone; however, within a few weeks developed sensory and motor symptoms in her left tibial nerve distribution. Symptoms stabilized with the addition of higher dose prednisone and methotrexate.

DISCUSSIONS:  CSS is a rare form of small vessel vasculitis of unknown etiology. It is classically characterized by asthma, tissue and blood eosinophilia, necrotizing vasculitis and a granulomatous response to eosinophilic necrosis. Rare nonasthmatic cases of CSS have been encountered in the literature as “atypical”, or “limited” forms of CSS. However, to the best of our knowledge, this is the first reported case in an individual without history of asthma manifesting the disease with generalized eosinophilic lymphadenopathy. This unusual presentation highlights the diagnostic challenge in CSS because of its protean manifestation. Furthermore, an early recognition is paramount as prompt treatment with corticosteroids can lead to rapid disease remission and limit morbidity in this otherwise progressive disease.

CONCLUSION:  This case illustrates the variable clinical manifestation of CSS. The lack of asthma or atopic history does not preclude the diagnosis. Lymph node involvement as part of systemic disease or as the primary site of involvement is rare but possible.

DISCLOSURE:  Julie Chou, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):22S. doi:10.1378/chest.136.4_MeetingAbstracts.22S-d
FREE TO VIEW

INTRODUCTION:  Diffuse alveolar hemorrhage (DAH) secondary to pulmonary capillaritis is an uncommon cause of hemoptysis in patients with primary antiphospholipid syndrome (APS). While APS is usually associated with thrombosis, we report a case of DAH, presumably related to pulmonary capillaritis, occurring after pulmonary thromboendarterectomy that resolved successfully with immunosuppressive therapy.

CASE PRESENTATION:  A 29 year old African-American female with chronic thromboembolic pulmonary hypertension related to antiphospholipid syndrome was referred for pulmonary thromboendarterectomy. She had a history of recurrent DVT and PTE from a young age, was chronically anticoagulated, and had an IVC filter. She underwent pulmonary thromboendarterectomy without immediate complications, and anticoagulation was resumed according to protocol. Ten days later, she developed massive hemoptysis requiring intubation and blood transfusion. Platelet count was 234, PTT was 28, and INR was 1.7. Initial bronchoscopy showed diffuse hemorrhage throughout both airways; however, a bleeding source could not be localized. CT angiography and pulmonary arteriogram did not show contrast extravasation or disruption of the pulmonary circulation. Bronchial artery embolization was performed on three occasions and transiently improved, but did not resolve, the hemoptysis. Repeat bronchoalveolar lavage (BAL), after failed embolizations, was consistent with DAH. Biopsy was deferred due to suspected active bleeding and increased FiO2 requirement. Serologies included a weakly positive P-ANCA at 1:80 with negative MPO and PR3, also consistent with DAH from presumed pulmonary capillaritis related to APS. Hematuria, absent upon admission, was evident. She ultimately received IVIG and pulse dose corticosteroid therapy. Hemoptysis resolved, and she was discharged on cyclophosphamide and corticosteroids. At 4 months post-op, there has been no bleeding recurrence despite her having resumed anticoagulation.

DISCUSSIONS:  DAH refers to a clinical syndrome resulting from injury to the alveolar capillaries, arterioles, and venules and may result from a multitude of disorders. It is characterized by hemoptysis, dyspnea, anemia, and bilateral alveolar infiltrates on chest radiography. The diagnosis is made by bronchoscopy, where serial BAL aliquots demonstrate persistent hemorrhagic fluid. Pulmonary capillaritis, a pathologic diagnosis, may cause DAH in some settings, usually related to systemic vasculitic syndromes like APS, Behcet’s, systemic lupus erythematosus (SLE) and Goodpasture’s. Primary APS, denoting APS that occurs in the absence of any other related disease, encompasses recurrent thromboses, both arterial and venous, and thrombocytopenia. Pulmonary manifestations of APS include multiple pulmonary emboli, pulmonary arterial thrombosis or microthrombosis with or without capillaritis, pulmonary hypertension, and alveolar hemorrhage. Antiphospholipid antibodies are not usually associated with hemorrhagic manifestations, and such an event occurring in an APS patient is usually due to deficiency of another coagulation factor, severe thrombocytopenia, severe uremia, or hepatic disease—none of which were evident in this patient. Indeed, pulmonary capillaritis was considered only after alternative diagnoses and therapies failed to resolve the bleeding. Treatment of DAH and pulmonary capillaritis in the setting of APS has not been evaluated in large clinical trials; however, case reports suggest that early use of cyclophosphamide and pulse dose corticosteroids may be beneficial.

CONCLUSION:  DAH from pulmonary capillaritis is an uncommon cause of hemoptysis in patients with primary APS. As demonstrated with this case, it should be considered in the differential diagnosis at any time for patients presenting with hemoptysis and an APS history. Early treatment with corticosteroids and immunosuppression may be effective.

DISCLOSURE:  Sonia Vishin, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):22S-e-23S. doi:10.1378/chest.136.4_MeetingAbstracts.22S-e
FREE TO VIEW

INTRODUCTION:  PAP was first described in 1958 and is of unknown etiology. Surfactant material fills the alveoli and stains positive with periodic acid-Schiff (PAS). The course of the disease ranges from respiratory failure to spontaneous resolution. It occurs in three clinical forms: congenital, secondary and acquired; the primary acquired disorder accounts for 90% of the cases. The acquired form may be an autoimmune disease targeting GM-CSF. About 500 total cases have been recorded in the literature with only 1 previously reported case of PAP associated with pregnancy.

CASE PRESENTATION:  36 years old woman, 25 weeks pregnant, presented with a 2 week history of increasing breathlessness and cough productive of whitish sputum. Her medical history: hyperlipidemia, tobacco abuse, DM and depression; medications: insulin and trazadone. During her last pregnancy in 2007, she presented with similar symptoms at 25 weeks gestation. Her CXR and CT chest at that time showed extensive bilateral ground glass opacities. She had negative: ANA, HIV, ANCA, Cocci serology and sputum GS and cultures. Bronchoscopy and BAL revealed intense PAS staining of amorphous bodies and granular material consistent with PAP. She underwent multiple total lung lavages and subcutaneous GM-CSF. Her PAP resolved after delivery of a normal fetus. Her current presentation was similar to that in 2007. She had an increasing O2 requirement of 12L to maintain saturation over 95 %. On examination she was cyanotic and tachypnoeic. There were normal pulmonary sounds on auscultation. Basic labs were unremarkable. CXR and CT chest showed the same bilateral alveolar and interstitial opacities with interlobular thickening. A presumptive diagnosis of PAP was made. Right lung lavage under general anesthesia was modified to RML lavage as patient had severe hypoxia requiring bag ventilation. The lavage fluid was initially turbid with sediment but gradually cleared. After the procedure, the symptoms diminished and oxygen saturation improved to 97% on 4L. Her PAS stain was again positive, microbiology was negative and LDH was 198. She was started on GM-CSF. She was also given dexamethasone for fetal lung maturity anticipating delivery before term. Last known she was 36 weeks pregnant and had only required one large volume lavage in the OR as well as weekly GM-CSF. She is to follow up with the pulmonary clinic after delivery to ensure resolution of her PAP.

DISCUSSIONS:  The pathophysiology of PAP is unclear but impairment of surfactant clearance by alveolar macrophages as a result of inhibition of the action of GM-CSF by blocking autoantibodies may underlie many acquired cases. BAL with PAS staining is sufficient to make a diagnosis. Acquired PAP has been treated successfully since the early 1960s by whole-lung lavage, and this procedure remains the standard of care today. Therapy with GM-CSF has shown promise in approximately half of those acquired cases treated, especially those with elevated LDH levels.

CONCLUSION:  PAP was of insidious onset in this patient’s last 2 of her 7 pregnancies with no clear etiology, which is the first recorded case report of this kind. We can only speculate on an association between changes in the maternal levels of GM-CSF during pregnancy and post-partum, and the development and subsequent resolution of the condition. We can substantiate this by our experience of significant clinical improvement seen in this patient with GM-CSF. Large volume lavage is both diagnostic and therapeutic and remains the standard of care.

DISCLOSURE:  Omer Ahmed, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):23S. doi:10.1378/chest.136.4_MeetingAbstracts.23S-c
FREE TO VIEW

INTRODUCTION:  Plastic bronchitis is a rare condition characterized by the formation of large endobronchial casts that obstruct the tracheobronchial tree. The inspissated casts obstruct the airways and can result in an acute life threatening airway emergency. Patients may require therapeutic bronchoscopy for cast removal. We describe the use of bronchoscopic cryotherapy in the removal of the airway casts in a patient with plastic bronchitis.

CASE PRESENTATION:  A 51 year-old male, life time nonsmoker, presented for evaluation of chronic cough productive of thick mucous plugs and dyspnea. The patient had severe bouts of dyspnea and required multiple hospitalizations, including intensive care unit monitoring for hypoxemic respiratory failure. A severe episode necessitated endotracheal intubation and mechanical ventilation. A chest radiograph showed opacification of the left hemithorax and computerized tomography of the chest confirmed occlusion of the left mainstem bronchus with collapse of the left lung. Flexible bronchcoscopy demonstrated tenacious secretions occluding the left mainstem bronchus. The casts were too thick to be suctioned through the bronchoscope and too friable to be grasped and removed with forceps. The use of fiberoptic bronchoscopic cryotherapy probe was used to remove a large left mainstem cast en bloc. This resulted in dramatic improvement in oxygenation and ventilation, and allowed for rapid extubation. Pathological analysis of the bronchial cast revealed acellular material consisting of fibrin and mucous with minimal inflammation and no eosinophils. A comprehensive evaluation for allergic bronchopulmonary aspergillosis, atopic diseases, cardiac disease, disorders of lymphatic drainage, and immunodeficiencies and vasculitidies was unrevealing. A clinical diagnosis of idiopathic plastic bronchitis was made. The patient required several bronchoscopies utilizing the cryoprobe to remove recurrent airway casts and relieve symptoms.

DISCUSSIONS:  The prevalence of plastic bronchitis is unknown and is likely under recognized as a disease entity. The most common associated diseases are cyanotic congenital heart disease, atopic conditions, and sickle cell acute chest syndrome. A new classification system of plastic bronchitis has been proposed and includes 1. structural congenital heart disease and mucin predominant casts, 2. lymphatic disorders and chylous casts, 3. asthma, atopy and eosinophilic casts, and 4. sickle cell acute chest syndrome with fibrinous casts. The natural history of plastic bronchitis depends on the associated disease and the type of cast. The treatment of plastic bronchitis includes bronchodilators, inhaled mucolytics, airway clearance techniques, and antibiotics. Other therapeutic options include inhaled heparin, dornase alfa and tissue plasminogen alpha. It may necessary to mechanically remove the airway casts if the patient presents with life threatening respiratory distress and hypoxemia. Urgent therapeutic bronchoscopy may be lifesaving. In our patient, cryotherapy allowed for the airway cast to be frozen and removed en bloc. This allowed the patient to be extubated and significantly improved gas exchange.

CONCLUSION:  Plastic bronchitis is an uncommon disease that can lead to acute endobronchial obstruction and life threatening acute respiratory failure. This case demonstrates the utility of bronchoscopic cryoprobe therapy to remove the large obstructing airway casts of a patient with plastic bronchitis.

DISCLOSURE:  Parvathy Nair, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):23S-d-24S. doi:10.1378/chest.136.4_MeetingAbstracts.23S-d
FREE TO VIEW

INTRODUCTION:  Bronchogenic cysts are rare congenital anomalies which present with a wide spectrum of clinical presentation from asymptomatic to acute respiratory distress. Radiographic manifestations are widely varied which sometimes make diagnosis difficult. We present a case where Endobronchial Ultrasound guided needle aspiration was both diagnostic and therapeutic.

CASE PRESENTATION:  37 year old female with 25 pack year of tobacco use, history of intravenous drug use and previous tuberculosis exposure treated with 9 months of isoniazid presented with fever, chills, chest pain and cough to her physician. She was treated with antibiotics for possible pneumonia as evident on chest radiograph. A follow up Computed Tomography (CT) after her symptoms did not resolve showed a 4 × 5.6 cm right paratracheal mass extending from the apex of the lung to the carina. She continued to have pleuritic chest pain although fever was improved. A repeat chest CT after 3 weeks showed persistent right paratracheal mass. She underwent bronchoscopy with attempted transtracheal needle aspiration at a local hospital, which was unsuccessful. The patient was then referred for possible surgical resection and biopsy of the undiagnosed mass. However, it was decided to do an endobronchial ultrasound (EBUS) guided 22G needle aspiration. About 150 milliliters of straw colored fluid was aspirated. Post-procedure chest radiograph showed significant reduction of the mass. Cytologic evaluation of the fluid yielded no malignant cells. Routine cultures grew upper airway contaminants, there was no purulence, acid fast culture and stain was negative.

DISCUSSIONS:  Bronchogenic cyst in adults occasionally presents as an asymptomatic radiographic finding unlike our patient who had constitutional symptoms. Conservative management with radiologic follow-up is controversial, given higher morbidity and mortality related to surgical excision after cyst growth. Definitive treatment of bronchogenic cyst is surgical excision given its high rates of recurrence and possible complications. However few case reports have described conservative management with intermittent transbronchial needle aspiration in patients with minimal symptoms or those reluctant or unable to undergo surgery. Our patient did not have any old chest imaging to confirm whether the cyst was present since her childhood, however, we entertained an infectious etiology with her intravenous drug use history and associated fever. All cultures including Acid Fast Bacillus (AFB) from the aspirated fluid were negative ruling out an infectious cause. She remains asymptomatic and we plan to follow her with serial imaging and reserve surgical excision only if it becomes symptomatic or recurrent. Occasionally these cysts may be loculated and a blind needle aspiration may not be completely successful. However, with EBUS guidance we can aspirate loculations of sufficient size.

CONCLUSION:  EBUS guided real-time drainage of bronchogenic cysts can be both diagnostic and therapeutic. Bronchoscopic needle aspiration with appropriate follow-up is a viable alternative to surgery in patients with mediastinal bronchogenic cyst. Surgical resection may be reserved for those with recurrent symptoms or if needle aspiration is unsuccessful.

DISCLOSURE:  Andrew Twehues, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):24S. doi:10.1378/chest.136.4_MeetingAbstracts.24S-d
FREE TO VIEW

INTRODUCTION:  Invasive aspergillosis is one of the commonest fungal infections in immunocompromised hosts, involving the respiratory tract in 90% of cases [1]. This disease occurs almost exclusively in immunosuppressed patients, although there have been few reports in immunocompetent patients. Invasive pulmonary aspergillosis (IPA), the most common manifestation of Aspergillus spp. infection in immunocompromised patients, typically involves the lung parenchyma, rarely affecting the trachebronchial tree exclusively.

CASE PRESENTATION:  A 65 year-old male with history of chronic lymphocytic leukemia developed cough and malaise eight months after an allogenic stem cell transplant. A computed tomography of the chest revealed an area of diffuse soft tissue thickening around the left main stem bronchus, which was intensely fluorodeoxyglucose-avid on positron emission tomography scanning (PET)(figure 1). An initial bronchoscopic exam revealed circumferential narrowing of the entire left main stem bronchus with necrotic and friable material on the medial wall. Both aspirates from this necrotic area and bronchial washing were not diagnostic. A second bronchoscopy with convex-probe endobronchial ultrasound evidenced a soft tissue thickening on the medial aspect of the left main stem bronchus underlying the area of necrosis visible endoluminally (figure 2). Endobronchial ultrasound-guided transbronchial needle aspiration performed in this area revealed multiple fungal elements suggestive of Aspergillus spp.

DISCUSSIONS:  Aspergillus fumigatus is the most common species responsible for invasive aspergillosis, followed by Aspergillus flavus, Aspergillus niger and Aspergillus terreus. Invasive aspergillus tracheobronchitis (IATB) is a rare manifestation defined as localized invasion of the bronchial wall by aspergillus. Young et al. reviewed the postmortem findings of 98 cases of aspergillosis finding the infection limited to the tracheobronchial tree in only five patients [2].Three morpholgical variants of IATB have been described: obstructive tracheobronchitis, ulcerative tracheobronchitis and pseudomembranous necrotizing bronchial aspergillosis. The ulcerative form,like the one we found in our patient, consists of lesions that penetrate through the tracheo-bronchial wall sometimes creating bronchoesophageal or bronchoarterial fistulas that may produce fatal hemorrhage. These three morphologic variants may indeed represent different stages in the development of invasive tracheobronchial aspergillosis.The insidious presentation of IATB with non-specific symptoms and the paucity of findings in chest roentgenograms often delay the diagnosis, giving this disease an ominous prognosis. There is little documentation of the radiologic features of IATB. We are presenting the first PET/CT scan images of IATB in the literature.The diagnosis of IATB is almost always confirmed by bronchoscopy. Aspiration of debris and bronchial washings allow the diagnosis in the majority of cases by showing the presence of aspergillus hyphae in special stains or recovering the organism in fungla cultures. To the best of our knowledge, this is the first case of IATB in which the diagnosis was facilitated by EBUS. It is our opinion that real-time EBUS might also be useful to delineate the depth of the tracheobronchial wall invasion and the involvement of major vascular structures, potentially preventing lethal hemorrhage.

CONCLUSION:  In conclusion, IATB is a rare form of invasive aspergillosis affecting mainly immunocompromised patients. The non-specific clinical presentation often leads to late diagnosis and a poor prognosis. We are reporting the first case of IATB diagnosed by EBUS-guided TBNA. We also cautiously suggest that EBUS imaging may be a useful tool to evaluate the degree of invasion and the involvement of vascular structures in these patients prior to bronchoscopic manipulation of the affected areas.

DISCLOSURE:  Roberto Casal, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):24S. doi:10.1378/chest.136.4_MeetingAbstracts.24S-e
FREE TO VIEW

INTRODUCTION:  Capsule endoscopy has become an important adjunct to identify obscure gastrointestinal bleeds and small intestinal disease. Contraindications to this procedure include intestinal obstruction and inability to swallow. Newer devices are now being utilized to decrease the risk of aspiration. We present a case of video capsule endoscopy resulting in pulmonary complications.

CASE PRESENTATION:  A 74 year old white male was transferred from an outside hospital for evaluation of gastrointestinal bleeding. The patient had both upper and lower endoscopy at the outside hospital with no bleeding source found. The patient had a past medical history of squamous cell cancer of the head and neck treated with right mandibulectomy, fibular flap reconstruction, tracheostomy which was later reversed, and radiation therapy. As a result, his ability to swallow was impaired and the patient was scheduled for direct endoscopic placement of a video capsule. During the procedure, the capsule along with the delivery housing was prematurely dislodged. The device was then incorrectly localized on radiography as being in the esophagus. The patient had no post procedure complications, complaints of shortness of breath, hoarseness or changes in his pulse oximetry. The next day, the patient had repeat upper endoscopy to localize the pill. The patient, however, was found to have an active duodenal bleed that was refractory to endoscopic therapy and was emergently taken to the operating room.Once intubated, the patient had prolonged episodes of hypoxia with PaO2 of 47mmhg on 100% oxygen. Intermittent suction resulted in transient improvements in hypoxia. After surgery for the duodenal ulcer, an emergency x-ray revealed complete opacification of the left hemithorax and the capsule in the left main stem bronchus. The pulmonary service was emergently consulted and bronchoscopy revealed complete obstruction of the left main stem bronchus by a video capsule. Due to its size and shape, the capsule proved to be too large for removal by basket, Roth net or forceps. Instead, a balloon catheter was expanded distal to the capsule and successfully used to dislodge it forward to the carina. ENT and cardiothoracic surgery were consulted for further assistance. Through the use of several different techniques, including suspension laryngoscopy and various inflatable catheters, the capsule was eventually removed by forceps from the oropharynx. The patient was transferred to the SICU in stable condition and later extubated without sequelae.

DISCUSSIONS:  This is a rare case of pulmonary complication from capsule endoscopy. To our knowledge, this is the first case associated with severe hypoxia or lung opacification and only the second involving aspiration to the left lung. Of the previous cases, 4 involved patients who eventually swallowed the capsule, 4 required bronchoscopic removal and one required retrieval from the cricopharyngeus. We hypothesize that our case was complicated by positive pressure ventilation used in the operating room that forced the capsule distally until it completely occluded the left main stem bronchus. The improvements in hypoxia from suctioning were likely due to ball-valve effect of the capsule.Although the capsule was dislodged from the left main stem via flexible bronchoscopy, complete removal of the device was not possible without further assistance. Due to the patient’s surgically altered upper airway, rigid bronchoscopy was difficult to perform. One previous case used a Roth net to retrieve the capsule, but because of the additional size of the housing surrounding our device, this was not feasible. Our case highlights the need for potential protocols or new techniques for bronchoscopic removal of video capsules or the need to alter the design of these devices for easier retrieval.

CONCLUSION:  Gastrointestinal complications, although rare, are the most common complications of capsule endoscopy. Our case highlights pulmonary complications from video capsule aspiration.

DISCLOSURE:  Adil Degani, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):25S. doi:10.1378/chest.08-1901
FREE TO VIEW

INTRODUCTION:  The incidence of bronchial stenosis after lung transplantation ranges from 1.6% to 32% and is the most common transplant airway complication. The stenosis may occur two to nine months after transplantation at the anastomotic site or distal to the anastomoses. Post-transplant bronchial stenosis are often treated with balloon bronchoplasty and silicone stent insertion, but in severe cases, may require surgical intervention.

CASE PRESENTATION:  A 61 year old male with a history of right single lung transplantation for severe COPD developed dyspnea with an associated decline in his spirometry 3 months post transplantation. Flexible bronchoscopy showed an 80% occlusive stenosis in the bronchus intermedius, immediately distal to the right upper lobe orifice. The right upper lobe bronchus also had an approximately 20% stenosis. Balloon bronchoplasty was performed with a CRE balloon, with immediate improvement in his dyspnea and spirometry; however he developed rapid recurrence of dyspnea. A rigid bronchoscopy was then performed with a repeat balloon bronchoplasty, rigid dilation, and silicone stent placement in the bronchus intermedius. The patient’s symptoms were immediately improved with an increase in his spirometry to baseline, but within one week, he returned with recurrent symptoms and a decrease in his spirometry. A repeat bronchoscopy revealed proximal dislodgement of his stent obstructing the right upper lobe entrance and worsening of the right upper lobe stenosis. The patient underwent another rigid bronchoscopy with the insertion of a silicone Y stent in the right main stem/right upper lobe/bronchus intermedius. All three limbs of the Y stent were shortened prior to insertion via rigid bronchoscope. After deployment of the stent, the flexible bronchoscope was used as a guide wire into the right upper lobe, concomitant with advancement of the stent with the rigid forceps into proper position. The patient’s symptoms subsequently improved and his spirometry returned to baseline. At three months follow-up he remained asymptomatic, he had a surveillance bronchoscopy which dislodged his stent and was removed. A similar sized Y stent was reinserted in the same position within the week without complications.

DISCUSSIONS:  Silicone stents are the preferred stent in the non-malignant airway. They offer the benefits of decreased granulation tissue, ease, and safety of removal. However silicone stents carry the risk of migration from the target location. The silicone Y stent may reduce migration by having an additional third limb to stabilize its position. The silicone Y silicone stent is used primarily for lower tracheal and main stem bronchus airway patency. To our knowledge, this is the first description of a silicone Y stent being used in the secondary carina for a post-transplant airway. This patient had a silicone Y stent in the secondary carina twice without complications during insertion. He maintained stable spirometry at the three months follow-up time point. In this case, the patient had additional distal stenosis beyond the anastomotic site; importantly a single silicone Y stent was able to maintain patency of two separate sites of bronchial stenosis.

CONCLUSION:  Silicone Y stents can be placed safely in a secondary carina for post-ansastamotic stenosis and may reduce stent migration complications.

DISCLOSURE:  Hans Lee, No Financial Disclosure Information; Product/procedure/technique that is considered research and is NOT yet approved for any purpose. Insertion of a silicone Y stent into the secondary carina.

Chest. 2009;136(4_MeetingAbstracts):25S. doi:10.1378/chest.136.4_MeetingAbstracts.25S-d
FREE TO VIEW

INTRODUCTION:  Aerodigestive fistula is a dreaded complication after treatment of esophageal carcinoma. The primary management method for patients with malignant disease is stenting in esophageus, airway or both. When technically feasible, surgery is the preferred treatment for benign disease. Herein, we report a case of bronchogastric fistula, occurring after esophagogastrectomy, managed with a combination of methods.

CASE PRESENTATION:  A 49 year old female presented with recurrent aspiration after placement of a metal esophageal stent for an esophageal stricture. She had a history of adenocarcinoma of the gastro-esophageal junction which was treated five years earlier with neoadjuvant chemoradiation and subsequent subtotal esophagectomy with Ivor-Lewis anastomosis. The patient developed an esophagogastric anastomotic stricture which required repeated dilatations. Despite multiple dilatations the patient remained symptomatic with solid dysphagia. She subsequently underwent placement of a 10 mm covered biliary stent. She tolerated that procedure well and continued to have improvement in her dysphagia. She then underwent removal of this stent and replacement by a larger 12 mm self expanding nonmetallic stent. The patient’s swallowing improved for a few months, but then she developed recurrent episodes of aspiration. Radiography revealed a fistula from her stomach to her left main bronchus. She then underwent bronchoscopy which revealed a 1 millimeter fistula in the posterior membrane of the left main bronchus 1 centimeter from the carina. Under rigid bronchoscopy a silastic stent was placed in the left main. She continued to experience aspiration after multiple replacements and revisions of the silastic stent. In February of 2009 she underwent rigid bronchoscopy with removal of the previously place silastic stent and placement of a 16 × 6 × 3 DJ (Diaz-Jimenez)® silicone prosthesis into the fistulous tract. That evening she unfortunately coughed forcefully which dislodged the DJ® silicone prosthesis. The following day she had replacement of the 16 × 6 × 3 DJ® silicone prosthesis and a 16 × 13 × 13 silastic Y-stent over the prosthesis to keep it in place. The patient had good palliation of her symptoms and no further episodes of aspiration. She has had no evidence of recurrence of her cancer.

DISCUSSIONS:  The DJ® silicone prosthesis is a new cufflink shaped silicon prosthesis that is inserted from the airway to occlude (in a clamshell fashion) a fistulous tract and prevent gastric content aspiration. It has been used in malignant tracheoesophageal fistulas for palliation as a novel approach to sealing an aerodigestive fistula. We report the successful use of this prosthesis in a non-malignant gastropulmonary fistula. In this case however the prosthesis became dislodged due to the size of fistula and a Y-stent was placed over the DJ® silicone prosthesis to secure it in place. This resulted in excellent symptomatic control for the patient.

CONCLUSION:  Stent migration is a frequent complication that can be difficult to manage. The DJ silicone aerodigestive prosthesis is a novel device used for closure of aerodigestive fistula. The combination of DJ stent and conventional airway stenting can be used to effectively and safely close fistula and prevent migration.

DISCLOSURE:  Jennifer Mattingley, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):25S-e-26S. doi:10.1378/chest.136.4_MeetingAbstracts.25S-e
FREE TO VIEW

INTRODUCTION:  The role of Continuous Positive Airway Pressure (CPAP) in asthma management has been well described (1). Higher positive end expiratory pressure is required to change baseline diameter of bronchus (2 ). Avoiding hyperinflation and high peak pressures have been the mainstays of mechanical ventilation in acute asthma. We describe a case of status asthmaticus responsive APRV.

CASE PRESENTATION:  A 21-year-old Caucasian female with history of moderate asthma presented to our Emergency Department with worsening dyspnea of four hours duration coupled with nonproductive cough, myalgias, coryza and rhinorrhea of three days duration. She described increasing dyspnea with wheezing requiring multiple emergency visits managed with standard therapy of antibiotics, bronchodilators and steroids over the preceding months. On presentation to our facility her only active prescription was Albuterol. She was initially admitted to general medicine with asthma exacerbation managed with bronchodilators, steroids and antibiotics. She deteriorated clinically and blood gas revealed an Arterial-alveolar gradient greater than 500 on 100% face mask and was admitted to the Intensive Care Unit. Investigations targeting shunt physiology were negative including computed tomography (CT) angiography of the chest and echocardiography with bubble study. She remained afebrile with mild leukocytosis and plain films of the chest revealed increasing basilar densities consistent with bibasilar ground glass seen on CT. The patient remained clinically unchanged despite aggressive medical therapy and was electively intubated on Day 3 to tolerate bronchoscopy with bronchoalveolar lavage (BAL) as imaging remained discordantly benign compared to the patients oxygen debt. She was placed on continuous positive airway pressure of 10 cm H2O with pressure support of 18 cm H2O with 80% FIO2 and saturations remained in the 85–95% range despite pulse dosing of steroids and continuous bronchodilator therapy. BAL revealed only mucous plugging with all cultures and stains negative for typical and atypical organisms. She began to have more frequent and sustained bouts of severe desaturation requiring escalating FIO2 and pressure support. The patient was cycled through multiple standard modes of ventilation with all being inferior to the patient’s clinical parameters on CPAP which was then, by Day 6, also failing. At that time the patient was placed on APRV with a P high /P low of 25/0 cm H2O and T high/T low of 5/0.85s generating a mean airway pressure of 21 cm H2O. Oxygen saturation rapidly increased to >95% and stabilized. Mean airway pressure was weaned deliberately to CPAP over a 24 hour period and the patient was durably extubated on hospital day 7.

DISCUSSIONS:  The use APRV in acute asthma has not been previously described. We document a rapid recovery in such a patient with the use of APRV. Xue et al demonstrated that hyperinflation of lungs with chronic CPAP decreases the airway responsiveness and contractile protein activation in airway smooth muscle (1). Whether or not this effect can be seen in acute settings with APRV is yet to be studied. It may be postulated that relief of air trapping with decreasing upper airway obstruction may be facilitated by the high MAP of APRV while the unique characteristics of APRV allow innocuous spontaneous respiration and, in our experience, a satiation of air hunger.

CONCLUSION:  Clinical guidelines for mechanical ventilation in acute asthma remain controversial. We believe that the distinctive mode of APRV in asthma management is under utilized. It is our hope that this report provides the impetus for further study.

DISCLOSURE:  Naveed Hasan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):26S. doi:10.1378/chest.136.4_MeetingAbstracts.26S-d
FREE TO VIEW

INTRODUCTION:  Recurrent respiratory papillomatosis (RRP) is a rare disease. In children vertical transmission of HPV is thought to be the causative agent. Although HPV has also been found in adults its route of transmission is less clear. The role of immune deficiency as a risk factor for disease development has not yet been determined. We will be presenting an adult patient with acquired immunodeficiency syndrome (AIDS) with RRP and distal dissemination.

CASE PRESENTATION:  A 64 year old male with AIDS (CD4 116) who presented with recurrent episodes of hemoptysis over a three month period. He also reported a productive cough, night sweats, and 15 lb weight loss. He had been ruled out for tuberculosis and treated for pneumonia during a recent admission for similar symptoms. On physical exam he was afebrile but had bilateral basilar rhonchi. Routine laboratory tests were unremarkable. Chest x-ray revealed patchy consolidation of the right mid and lower lobes with CT chest confirming these areas of consolidation as well as revealing a large endotracheal lesion. PET CT showed increased uptake in the trachea and areas of consolidation. Subsequent fiberoptic and virtual bronchoscopies demonstrated numerous exophytic polyps lining the entire trachea down to the carina. Histology was positive for squamous papilloma fragments and the presence of low risk strains of HPV was confirmed by HPV-DNA testing. Upon further review we found that he had first been diagnosed with tracheal squamous papillomas two years prior but unfortunately was lost to follow-up while caring for his wife who had been diagnosed with cervical cancer. During this admission there had been significant progression of the papillomas and distal dissemination and malignant transformation were concerns. CT-guided RLL lung biopsy revealed dysplastic squamous epithelium with foci of squamous metaplasia confirming our diagnosis of RRP with distal parenchymal dissemination.

DISCUSSIONS:  RRP is a rare disease. Although more commonly diagnosed in children with airway obstruction there have been reported adult onset cases. In children vertical transmission of HPV is thought to be the causative agent. Although in adults HPV has also been found in these lesions the route of transmission is less clear. Oro-genital sexual transmission seems like the most likely culprit with low risk HPV strains 6 and 11. Treatment involves surgical laser resection. Other options include interferon-alpha, antivirals, cimetidine, and celebrex all of which are being studied. Even with surgical treatment lesions tend to recur. Most will require multiple surgical interventions to control disease complications. Distal spread has been reported into the smaller bronchial airways and lung parenchyma. Malignant transformation into squamous cell carcinoma is also a major concern. PET CT does not seem to be useful for accessing malignant transformation since increased uptake is also present in the paillomas. Currently no recommended screening guidelines exist. Another area of uncertainty is whether AIDS itself is a risk factor for RRP. There has been a case report of disease development after immune reconstitution with highly active antiretroviral therapy (HAART). However our patient had low CD4 counts and known noncompliance with HAART. We propose that this patient’s immune deficiency increased his risk for development of RRP with distal dissemination.

CONCLUSION:  1. RRP is caused by low risk HPV stains.2. RRP may present with endotracheal lesions and distal dissemination may present as pneumonic infiltrates. 3. PET scan is not useful in accessing malignant transformation. 4. In adult onset RRP AIDS may be a risk factor.

DISCLOSURE:  Ami Abraham, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):26S. doi:10.1378/chest.136.4_MeetingAbstracts.26S-e
FREE TO VIEW

INTRODUCTION:  Tracheobronchomegaly (TBM), also know as Mounier-Khun syndrome, is a rare disorder characterized by marked dilation of the trachea and main bronchi occasionally complicated by tracheal diverticulosis, bronchiectasis and recurrent lower respiratory tract infections. Presentation varies from incidental radiographic finding to progressive respiratory failure.

CASE PRESENTATION:  A 46-year-old male was referred with diffuse “cystic” lung lesions discovered on chest tomography (CT). He initially presented with lower respiratory tract infection requiring 14-day course of intravenous antibiotics 6-months earlier. The patient was a 27-pack-year tobacco smoker, quit 2 years prior to evaluation. He carries no significant medical or surgical diagnoses with exception to hypercholesterolemia requiring a statin therapy over the past 4 years. Review of systems revealed a 2-year non-progressive exertional dyspnea. He denied excessive mucous, cough, recurrent infections, or childhood pulmonary disorders. He further denied weight fluctuation, loss of appetite or night sweats. Furthermore, he denied joint or auricular inflammation, ophthalmologic disorders or recurrent sinusitis. He has fathered 4 children. He denied alcohol, illicit drug, HIV risk factors or suggestive occupational or environmental exposures. Physical examination revealed a chronically thin, well-nourished African American male with a body mass index of 18.8. His vital signs were within normal limits with an oxygen saturation of 97% while breathing ambient air. His lung examination revealed no obvious abnormalities. Rest of exam was normal. Spirometry showed no evidence of airflow obstruction and remained stable since his earlier study 6-months ago. Diffusion capacity for carbon monoxide showed a mild impairment (21.9 mL/mmHg/min, 74% predicted). Review of his chest roentogram revealed tracheomegaly (41 mm) without other obvious abnormalities (Fig. 1). In transaxial images, CT of his chest further confirmed the tracheomegaly in addition to bilateral bronchomegaly (Right: 25.4 mm; Left 23.4 mm) and diffuse varicose bronchiectasis extending to the fifth-order of bronchial division (Fig.2. Arrow). A closer examination of his chest CT showed proximal tracheal diverticulosis. Laboratory findings showed normal alpha-1-antitrypsin level, sedimentation rate, ANA, anti-SSA and SSB. Immunoglobulin levels (IgG) and related subclasses were within normal limits. Total IgE and IgE-specific for aspergilus were normal. Sputum culture showed no acid-fast bacteria and was negative for mycobacterium avium complex (MAC). Genetic testing for Cystic Fibrosis showed no obvious mutations.

DISCUSSIONS:  Mounier-Kuhn syndrome is primarily a radiographic diagnosis in the correct clinical setting. The etiology remains uncertain, however evidence suggest a congenital defect or atrophy of the elastic and smooth muscle tissue of the trachea and main bronchi. Though a scant amount of literature points to a familial, recessive pattern of inheritance, the majority of cases appear to be sporadic. The disease predominantly occurs in middle age men with African American descent. Radiographically, any diameter of the trachea, right main and left main bronchus that exceeds 3.0 cm, 2.4 cm and 2.3 c, respectively, on a standard chest radiograph is diagnostic of TBM. For chest CT, the values are 3.0, 2.9, and 1.8, respectively. Treatment is limited to physiotherapy to assist in secretions and appropriate antibiotics in times of exacerbation. Scarce case reports of lung transplantation and tracheal stenting for tracheal stenosis have been reported with variable outcomes.

CONCLUSION:  TBM is a radiologic diagnosis in the context of a fitting clinical presentation. Immunizations, clearance of secretions and appropriate duration and choice of antibiotics are crucial in management. Being familiar with the radiographic findings could potentially prevent unnecessary surgical and/or endoscopic evaluation. Spirometric pattern in patients with TBM is difficult to predict, however, our case is the second in the literature documenting normal spirometric findings.

DISCLOSURE:  Mohammad Omari, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):27S. doi:10.1378/chest.136.4_MeetingAbstracts.27S-d
FREE TO VIEW

INTRODUCTION:  Vascular rings are uncommon congenital malformations that present with dysphagia or upper airway obstruction and are well described in the pediatric cardiology literature. Tracheal compression by the innominate artery is considered an “incomplete” vascular ring, and is a rare diagnosis of this category. The innominate artery crosses anterior to the trachea where it can cause compression and lead to stridor, respiratory distress, and recurrent lower respiratory tract infections. Typically symptoms are prominent in infancy but improve by the second year of life. To our knowledge, tracheal compression by the innominate artery has not been described presenting in adult population.

CASE PRESENTATION:  70-year-old West African woman presents to our hospital with dyspnea on exertion and left-sided chest pain. She is admitted for cardiac catheritization. Patient has been carrying a diagnosis of chronic obstructive pulmonary disease (COPD) for multiple years. She has had history of only second hand smoke exposure and has been treated with albuterol and ipratropium. Her other medical history included hypertension, hyperlipidemia, coronary arterial disease requiring coronary stent placement for acute myocardial infarction, kyphosis, and a ventral hernia. Her physical exam was notable for stridor, but polyphonic wheezing was not appreciated throughout. Furthermore, chest radiograph imaging was not consistent with the diagnosis of asthma or COPD. A high-resolution, multislice computed tomographic (CT) scan revealed a tortuous innominate artery indenting and significantly compressing the trachea. The patient had considerable kyphosis, which was thought to contribute to the tracheal compression presenting so late in life by causing changes in thoracic anatomy. The vascular surgery team was consulted. The patient was deemed a poor surgical candidate due to her advanced age and comorbidities, especially given the relatively stable nature of tracheal compression.

DISCUSSIONS:  Tracheal compression from an innominate artery arising from a normal left-sided aortic arch is a rare phenomenon. It was first described by Gross and Neuhauser in 1948 in an infant presenting with cough, stridor and apnea. Numerous pediatric cases have since been reported, some with symptomatic relief after decompressive surgery. Strife et al found that up to 30% of children have some anterior indentation of the trachea without symptoms, and in one series only 39 of 285 symptomatic cases required surgery. Medical management includes humidified oxygen, steroids and antibiotics, while common surgical approaches include arteriopexy, with suspension of the innominate artery to the sternum, or reimplantation at a more proximal site of the ascending aorta. Typically symptoms of tracheal compression gradually improve in the first two years of life with the development of the trachea’s cartilaginous rings, and cephalic, rightward, anterior movement of the innominate artery away from the trachea. Myer et al reports of innominate artery compression of the trachea in adolescents, further suggesting that flow volume loops are an effective method for measuring compromise and that exercise intolerance and apnea spells are possible indications for surgical repair. Tracheal rings have rarely been newly diagnosed in adults. Several authors have reported them in adult patients presenting with a right aortic arch. However, we could not find any report of tracheal compression caused by the innominate artery presenting in adulthood.

CONCLUSION:  This case of a 70-year-old woman with a rare tracheal compression demonstrates the importance to cast a wide differential when working up shortness of breath, especially when the history and physical do not match well with a common diagnosis. In this case an early correct diagnosis could have resulted in a possibility for a corrective decompressive vascular surgery with less morbidity.

DISCLOSURE:  Louis Gerolemou, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):27S-e-28S. doi:10.1378/chest.136.4_MeetingAbstracts.27S-e
FREE TO VIEW

INTRODUCTION:  Hemoptysis is an uncommon complication of pregnancy. We report a case of a young woman with Lady Windermere syndrome and chronic asthma with recurrent pregnancy related hemoptysis.

CASE PRESENTATION:  A 37 year old female (G2P1L1) in her 14th week of twin pregnancy presented with hemoptysis and shortness of breath for one week. The patient reported 1 teaspoon of bloody expectoration 2–3 times a day for the past week. There were no fever chills or cough reported prior to the episode of hemoptysis nor recent travel or sick contacts. The patient had similar episodes of non-massive hemoptysis during the last trimester of her first pregnancy, that resolved with conservative management. She has past medical history of well controlled asthma and recurrent childhood pneumonia with resultant Lady Winderemere syndrome. She has had no history of hemoptysis prior to the first pregnancy and between the pregnancies. Vital signs on admission were normal. Physical examination revealed focal coarse crepitations over the anterior right chest wall and bilateral expiratory wheezing. Cardiac and extremity examination were within normal limits. Abdominal exam was consistent with the 14th week of pregnancy. CXR showed increased markings in the region of right middle lobe.CT scans obtained after her first pregnancy revealed localized right middle lobe bronchiectasis. She was admitted for hemoptysis and asthma exacerbation. She was treated with albuterol and ipratropium nebulzations and a short course of oral corticosteroids. During the hospital stay she had few more episodes of hemoptysis that resolved with conservative treatment. The dyspnea improved and was discharged on as needed inhaled albuterol and inhaled steroids.

DISCUSSIONS:  Lady Windermere syndrome is a constellation of right middle lobe infiltrate in the setting of chronic Mycobacterum avium-intracellulare infection. The clinical manifestations of this syndrome consist of chronic cough, sputum production and occasional hemoptysis. The physiologic changes associated with pregnancy leads to an increase in blood volume, cardiac output and generalized vasodilatation. This has been associated with hemoptysis in patients with pulmonary vascular malformations. This is the first reported case where these changes are associated with hemoptysis in patient with Lady Windermere syndrome. We hypothesize that the recurrent episodes of hemoptysis manifesting only during pregnancy were secondary to the effect of these physiological changes on the areas of bronchiectasis. The fact that the hemoptysis occurred earlier in the second pregnancy which was the twin gestation strengthens the proposed hypothesis.

CONCLUSION:  Hormonal and hemodynamic changes of pregnancy can cause hemoptysis in patients with bronchiectasis.

DISCLOSURE:  Prashant Gundre, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):28S. doi:10.1378/chest.136.4_MeetingAbstracts.28S-c
FREE TO VIEW

INTRODUCTION:  Tracheobronchopathia Osteochondroplastica (TPO) is a rare disorder characterized by submucosal calcified nodules. Several case reports have described its presentation. However, long term prognosis in TPO is poorly understood. We present five cases of TPO with follow up evaluations at varying intervals.

CASE PRESENTATION:  Our cases included four women and one man. Mean age was 68.2 years (59–72) at time of diagnosis. All presented with cough, post-nasal drainage, or gastric reflux symptoms. One patient recently started an angiotensin enzyme inhibitor and presented with mild hemoptysis. Pulmonary function tests were normal in three patients, obstructive in one, and restrictive in one. All patients underwent bronchoscopic evaluation revealing round, hard, sumbucosal nodules with normal overlying mucosa. Follow-up examinations and CAT scans were performed on all patients. None of the patients had evidence of advancing disease. Two of the patients were followed for greater than six years. Neither patient had progression of symptoms or nodules as seen on virtual bronchoscopy.

DISCUSSIONS:  TPO is a rare disorder of unclear etiology; however, abnormal bone morphogenetic protein 2 may play a role in initiating calcium deposition. Case series suggest 88 percent of patients have upper respiratory symptoms and cough at presentation. Diagnosis is made by direct visualization of characteristic lesions under bronchoscopy. In many cases, symptoms resolve but reported complications include airway obstruction, post-ostructive pneumonia, recurrent pneumonia, atelectasis, and difficult intubation. Rare cases of progressive disease have been reported. These patients were treated with laser ablation or tracheostomy. Treatment is usually directed at underlying disorders such as chronic obstructive pulmonary disease, asthma, bronchiectais, gastric reflux disease or upper respiratory cough syndrome. Minimal literature exists describing the long term behavior of TPO lesions or optimal follow-up. In our patients, the lesions did not change on follow up virtual bronchoscopy, suggesting TPO may be a reactive process to a single event leading to permanent changes.

CONCLUSION:  TPO is a rare usually benign disorder; however, progressive disease has been reported. The etiology and long term behavior of TPO lesions are poorly understood. Treatment is directed at comorbid respiratory disorders that cause chronic cough. As our images show, virtual bronchoscopy offers a potential non-invasive method of diagnosis and follow-up imaging.

DISCLOSURE:  Scott Hagedorn, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):28S. doi:10.1378/chest.136.4_MeetingAbstracts.28S-d
FREE TO VIEW

INTRODUCTION:  It’s a point of privacy to the health institutions to avoid reporting laboratory acquired infections, especially with the Absence of a formal forum outside the institution, and absence of obligatory mechanism for reporting that over 500.000 workers in the USA alone are employed in laboratories dealing with wide range of microorganisms with a potential for direct transmission.

CASE PRESENTATION:  A 65 year old Indian male presents to the Employee Health Department with productive cough and hemoptysis for the last two weeks. He also complains of anorexia, fatigue and weight loss for the last 6 months. Review of systems was unrevealing. He has no past history of chronic illness. He is on no medications except for omeprazole and triamcinolone cream. He has no sick contacts nor was he exposed to TB. Socially he is married and has been living in Ohio for fifteen years since he moved from Houston, TX where he lived for 3 years, he has no history of recent travel outside the area. He does not smoke, drink alcohol nor use illicit drugs. Works as a lab technician dealing mostly with Coccidioides for the past 10 years, participating in a research projects. Denies any accidents or breaks of biosafety rules in level II research lab. Basic labs tests normal.Chest Xray showed increased fibrotic markings and a left upper lobe nodule (fig. 1). PPD skin test was negative as well as sputum acid fast bacilli. Coccidioidomycosis skin test and antibodies were positive.A CT of the chest demonstrated extensive bronchiectasis in the right middle lobe; calcified lymph nodes in the mediastinum and cavitating lesions in the apices bilaterally (fig. 2). Transbronchial biopsy and Mycobacterial cultures were negative. The fungal cultures revealed the classic arthroconidia (fig. 3). Patient was started on Fluconazole. Shortly after he started to feel better and to gain weight.

DISCUSSIONS:  Coccidioidomycosis is a systemic infection caused by Dimorphic fungus Coccidioides immitis, initially recognized by Posada 1892, Coccidioides spp. is endemic to lower sonoran deserts of the western hemisphere including northern Mexico, southern Arizona, central and southern California, and westTexas, under normal conditions person to person transmission does not occur, apporoximately 100,000 infections occur in the united states each year, incidence of infection in endemic areas like Arizona is 0.43% with higher risk for pregnancy, immunosuppression, African American and Fillipinos . The laboratory hazard of this organism is related to its size (2–5 millimicrons), the arthroconidia are conducive to ready dispersal in air and retention in the deep pulmonary spaces. The much larger size of the spherule (30–60 millimicrons) considerably reduces the effectiveness of this form of the fungus as an airborne pathogen. Inhalation of arthroconidia from environmental samples or cultures of the mold form is a serious laboratory hazard. The CDC recommends Biosafety Level 2 practices and facilities for handling and processing clinical specimens, identifying isolates, and processing animal tissues. Animal Biosafety Level 2 practices and facilities are recommended for experimental animal studies when the route of challenge is parenteral, and Biosafety Level 3 for propagating and manipulating sporulating cultures already identified as C. immitis and for processing soil or other environmental materials.

CONCLUSION:  Interestingly, this is the first reported laboratory acquired coccidiomycosis since 1978. since the implementation of more meticulous biosafety rules. We believe laboratory acquired Coccioidomycosis both under recognized and under reported.

DISCLOSURE:  Ziad Mattar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):28S-e-29S. doi:10.1378/chest.136.4_MeetingAbstracts.28S-e
FREE TO VIEW

INTRODUCTION:  Opportunistic infections are commonly detected in patients with Acquired Immuno-Deficiency Syndrome (AIDS). The most common infective pathogen accounting for focal brain lesions in AIDS patients is Toxoplasma gondii. However, due to the lack of an adequate immune system, other pathogens must be considered along with a complete assessment of the patient’s social history when making a diagnosis.

CASE PRESENTATION:  38 year old Honduran woman with no known medical history was admitted to the hospital after presenting to the emergency department with progressive right-sided hemiparesis, myalgias and headache. Computed tomography (CT) scan of the head showed areas of vasogenic edema in the left fronto-parietal and right parietal regions. Due to the concern for an infectious process a lumbar puncture was performed. The opening pressure was 11 cm of water, with 83 red blood cells, no white blood cells, glucose of 45 mg/dl, total protein of 29.2 mg/dl and negative microbiological studies and stains. Human Immunodeficiecy Virus (HIV) testing was positive with a subsequent CD4 count of 104 cells/mm3. Magnetic resonance imaging (MRI) of the head revealed numerous bilateral ring-enhancing lesions with necrotic centers with the largest measuring approximately 4 × 2.8 cm. Testing for Toxoplasma IgG and IgM, Crytococcus neoformans antigen, Histoplasma capsulatum antigen, Herpes Simplex Virus, Cytomegalovirus and Acid-fast bacilli (AFB) were all negative. Neurosurgery was consulted and the patient was then taken to the operating room for a brain biopsy. Additional stains were performed on the brain biopsy included for EBV (Epstein-Barr virus), AFB and cryptosporidium. The preliminary result communicated from pathology was consistent with Histoplasmosis, but more stains were needed due to atypical morphology and large amount of organisms seen. Following the brain biopsy the patient suffered a seizure and was transferred to the medical intensive care unit for management of cerebral edema and possible herniation. The patient was endotracheally intubated and placed on mechanical ventilation for airway protection and hyperventilation. Repeat CT of the head at that time showed no evidence of herniation but significant progression of the lesions. Final pathology results revealed necrotizing encephalitis with abundant organsisms consistent with Trypanosoma cruzi, which was confirmed by the Centers of Disease Control. The patient was then started on nifurtimox. The patient was eventually weaned off the ventilator and extubated. Subsequent repeat CT of the head showed stability of the lesions and mild improvement of the cerebral edema. The patient was transferred back to the medicine ward, but remained non-communicative with no improvement in mental status or hemiparesis.

DISCUSSIONS:  Our patient’s presentation appeared consistent with cerebral toxoplasmosis, especially in the setting of undiagnosed HIV/AIDS. However, because progressive hemiparesis is not typical of toxoplasmosis, along with an otherwise negative work-up, a brain biopsy was required to make the final diagnosis of cerebral trypanosomiasis. Seropositivity for trypanosomal infection is well known among the people of endemic regions. Among immigrants from endemic regions there is high seroprevalence of chronic Chagas without clinical symptoms. Among 205 Central American immigrants in the Washington D.C. area tested between 1984–1985, 4.9% were infected with T. cruzi and parasites were isolated in 50% of attempted xenodiagnostic analysis. Although cardiac and gastrointestinal involvement are the main manifestations in the general population, cerebral infection is a well documented site for reactivation of chronic disease among the immuno-compromised patients, specifically those with AIDS.

CONCLUSION:  Cerebral toxoplasmosis continues to be the most common infectious cause in AIDS patients with a cerebral lesion. In endemic patients, Trypanosmiasis must be considered and tested for as a possible causative agent when more common tests are non-diagnostic.

DISCLOSURE:  Alberto Colomer, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):29S. doi:10.1378/chest.136.4_MeetingAbstracts.29S-d
FREE TO VIEW

INTRODUCTION:  Pulmonary blastomycosis resulting in acute respiratory distress syndrome (ARDS) has a very high mortality in spite of appropriate treatment with anti-fungals. Case reports utilizing adjunctive corticosteroids in ARDS due to blastomycosis suggest benefit. We present a case of ARDS due to blastomycosis treated with corticosteroids early in the disease course resulting in rapid improvement.

CASE PRESENTATION:  A 19 year old female, resident of Indiana was transferred to our tertiary care center for evaluation of dyspnea and bilateral pulmonary infiltrates. She reported fevers, dyspnea and cough for 3 weeks. She was admitted to a local hospital and was treated with ceftriaxone and azithromycin but continued to worsen. As a result, the antibiotic coverage was changed to piperacillin-tazobactam, trimethoprim-sulfamethoxazole, linezolid and itraconazole. CT scan of the chest showed diffuse bilateral infiltrates. A bronchoscopy with bronchoalveolar lavage was non-diagnostic. She continued to deteriorate with increasing oxygen requirements. In our facility, a CT guided fine needle aspiration of left lower lobe infiltrates was performed which showed multiple broad-based budding yeast, consistent with Blastomyces dermatitidis. Because of her increasing oxygen requirements and worsening lung infiltrates she was intubated and mechanically ventilated. A diagnosis of ARDS was made, based on bilateral pulmonary infiltrates and a PaO2/FiO2 ratio of 156. Low lung compliance required low tidal volume per ARDS.net protocols. She was started on methylprednisolone (50 mg IV q6hours) and liposomal amphotericin B (520 mg/day). Her oxygenation and lung mechanics improved dramatically and she was extubated after 4 days of mechanical ventilation. She continued to improve, steroids were tapered, amphotericin was switched to oral itraconazole, and she was discharged to home one week later.

DISCUSSIONS:  Acute respiratory distress syndrome is an infrequent manifestation of blastomycosis infection. It has been associated with high mortality rates reaching 89%, even with appropriate anti-fungal therapy (1). According to the Infectious Diseases Society of America guidelines, Amphotericin B is the treatment of choice for severe diffuse pulmonary blastomycosis. Some mycologists advocate the additional use of corticosteroids in cases of ARDS secondary to blastomycosis. It has been postulated in a prior report (2) that blastmycosis induces a hyperinflammation syndrome and that adjunctive steroid therapy will reduce this inflammatory response analogous to that seen in Pneumocystis jiroveci pneumonia. In that report, good outcomes were demonstrated for two patients with severe ARDS secondary to blastmycosis who were treated with corticosteroids. We elected to add corticosteroids early in the disease course in an attempt to curb the extent of hyperinflammation associated with severe pulmonary blastomycosis.

CONCLUSION:  ARDS secondary to blastomycosis carries mortality approaching 90%. The role of corticosteroids is still uncertain in the treatment algorithm of ARDS in general, but there are certain sub-groups where corticosteroids are of definite benefit. Based on our report, ARDS induced by blastomycosis may be one of them.

DISCLOSURE:  Babar Khan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):29S-e-30S. doi:10.1378/chest.136.4_MeetingAbstracts.29S-e
FREE TO VIEW

INTRODUCTION:  We present a case of a 29 year old Caucasian male with an intermittent productive cough of 3 years duration with focal bronchiectasis.

CASE PRESENTATION:  He is a seafood chef with no significant past medical history except for extensive travel throughout Asia and Africa with multiple ingestions of raw seafood. He was in his usual state of health until 3 years ago when he developed a productive cough with associated hemoptysis. A CT scan of the chest was obtained which revealed consolidation of the right middle lobe and superior segment of the right lower lobe with bronchiectasis. An evaluation by an outside pulmonologist led to several courses of oral antibiotics and two bronchoscopies for persistent symptoms. The only positive cultures from the bronchoscopies yielded Haemophilus influenzae and enterobacter cloacae. However, despite multiple regimens of antibiotics he remained symptomatic with a productive cough and occasional hemoptysis. He was referred to our institution and we evaluated his bronchiectasis with a sweat chloride test, aspergillus IgE levels, sputum cultures, as well as alpha one anti-trypsin level. None of the tests were diagnostic, and he underwent a bronchoscopy which revealed normal airways and subsequent cultures grew normal respiratory flora. He continued to have a persistent productive cough and was ultimately referred for a lobectomy for focal bronchiectasis. The tissue biopsy revealed bronchiectasis with granulomas formed around polarizable parasite eggs. A cross section of the sample revealed a worm suggestive of P. Westermani. He was treated with a short course of praziquantel with eventual resolution of symptoms.

DISCUSSIONS:  Often the initial symptoms of paragonimiasis infection are epigastric with abdominal pain correlating with the migrating larvae stage. As the larvae enter the pleural cavity, symptoms consistent with pleurisy may be observed. Radiographically, there is often bilateral involvement, with or without pleural effusion, but may be normal in 10–20% of people. The clinical symptoms correlating with the migration of the worm through the lung include cough, chest pain, malaise, and hemoptysis1. Associated images on x-ray will reveal migratory, transient infiltrates with no specific lobar predilection. As the worm matures, the patient may have recurrent hemoptysis, often chocolate colored and with a distinct odor. The mature worms settle down and cysts form around them2 . The mechanism is thought to be obstruction of the arteriole or venule by the eggs of the worm, which leads to ischemic infarct. There is then an expansion of the small airway by the intraluminal parasite. The cyst can be filled with hemorrhagic fluid and thus appear as a mass-like consolidation. There have been described tubular structures that communicate with the cysts. These either represent bronchiectatic airways or the worm tracks laid down as the result of the migration2 . The main CT finding of pleuropulmonary paragonimiasis have been described as air space consolidation and pulmonary nodules1. Consolidations are thought to represent the early stages of the disease, whereas nodules, cysts, and bronchiectasis are thought to represent the later stages of the disease.Our patient had CT findings of mild, localized bronchiectasis which was later confirmed grossly. We postulate that the recurrent infections were in part due to obstruction caused by the intraluminal parasite or its eggs. The subsequent recurrent infections and inflammation likely resulted in the eventual destruction of the airways as he had no other risk factors for bronchiectasis. The source of the infection was determined to be due to the patient’s consumption of live crabs.

CONCLUSION:  This case represents an unusual cause of bronchiectasis. In patients with the appropriate history, paragonimiasis infection should be included in the differential diagnosis for bronchiectasis.

DISCLOSURE:  Ali Massoumi, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):30S. doi:10.1378/chest.09-0637
FREE TO VIEW

INTRODUCTION:  Strongyloidiasis is a parasitic disease caused by Strongyloides stercoralis (SS), a soil transmitted nematode of world wide distribution. This is a case report of a non-smoking farmer from Guyana who emigrated to the USA fourteen years ago. Over the course of 5 years she had recurrent hospitalization for AIHA, persistent wheezing, exertional dyspnea and eosinophilia.

CASE PRESENTATION:  Ms. SN is a eighty one year old female with past medical history of AIHA, B12 deficiency,aortic stenosis and chronic renal insufficiency. She was admitted in November 2004 with progressive wheezing, exertional dyspnea, cough productive of yellow sputum and fever for two weeks.She was diagnosed with B12 deficiency two and a half years ago. She also had recurrent hospitalizations for wheezing and exertional dyspnea over the same period. Work up revealed positive direct coombs test, raised Ig G, ANA of 1:320 and haptoglobin of <6. AIHA was diagnosed and corticosteroid was commnenced 4 months ago. Anemia persisted despite B12 supplimentation and corticosteroid treatement. She had multiple transfusion of packed red blood cells. Wheezing and exertional dyspnea progressively worsened until she became house bound. There was also a steady increase in eosinophil count. Continued administration of bronchodilators and corticosteroid provided no relief to these symptoms. On admission her blood pressure was 138/63 mmHg, heart rate was 66 beats/minutes, respiratory rate 24 breaths/minute, temperature 98.4 degrees Fahrenheit, oxygen saturation 93% on room air (RA) and peak flow 140 L/minute. Examination revealed an obese female with diffuse expiratory wheeze and a grade 2/6 systolic murmur over the precordium. Her hemoglobin was 6g/dL. She had a normal WBC with 35% eosinophils. Her blood urea nitrogen and creatinine were 29 and 1.8 respectively. Her arterial blood gas at RA was pH 7.33, PCO2 38, PO2 68. Chest x-ray showed enlarged cardiac silhouette. Echocardiography, electrocardiogram and ventilation perfusion lung scan were normal. She was transfused with two units of packed red blood cells and was treated with bronchodilators, corticosteroid and clarithromycin with little effect on wheezing and exertional dyspnea . Further work up revealed an elevated Ig E of 2600 (normal < 114), anti-filarial Ig G antibody titer of 1:4, positive stool SS larvae and moderate restrictive defect with DLCO of 52% on pumonary function test. A single dose of Ivermectin 200mcg/kg was administered. Within one week her dyspnea and wheezing resolved and over the ensuing three months, the eosinophilia and increased Ig E almost normalized. She no longer required bronchodilators, corticosteroid or B12 supplemetation.

DISCUSSIONS:  Refractory wheezing, with restrictive or mixed ventilatory defect due to SS is an uncommon and under-diagnosed condition. In immunosuppresed populations, escalating to higher doses of corticosteroid in desperate attempt to control wheezing may lead to disastrous consequences. Positive filarial antibodies is not always diagnostic of tropical eosinophilia since cross reactivity of filarial and SS antibodies does occur. AIHA however is an exceedingly rare manifestation of SS. To the best of our knowledge the only published case of hemolytic anemia in association with SS was not documented as coombs positive. As in our patient it may be appropriate to treat strongyloides initially with follow up reassessment for additional filariasis treatment. Furthermore, Ivermectin in multiple doses has been shown to be effective in eradication of microfilaria as well.

CONCLUSION:  SS infestation acquired years earlier, may manifest as hyperinfection syndrome decades later under the influence of immunosuppressive medications. SS infestation should be considered in the differential diagnosis of AIHA especially when associated with eosinophilia and refractory respiratory symptoms.

DISCLOSURE:  Alfred Ajise, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):30S-d-31S. doi:10.1378/chest.136.4_MeetingAbstracts.30S-d
FREE TO VIEW

INTRODUCTION:  Leptospirosis is a zoonotic disease usually found in developing nations, but infrequently encountered in the U.S. We report the case of a returning traveler with multi-organ dysfunction found to have Weil’s disease, the most severe form of leptospirosis.

CASE PRESENTATION:  A 38 year-old healthy male presented to a local emergency department complaining of a five day history of fevers, chills, malaise, frontal headaches, and dark-colored urine. The patient had previously been vacationing in northern Jamaica about 4 weeks prior to presentation, where he had spent time swimming in local lakes. CXR was notable for an infiltrate and he was started on ceftriaxone and azithromycin. He was subsequently transferred to UAB, where initial oxygen saturation on room air was 85% and physical exam was notable for jaundiced skin and icteric sclera. Labs on transfer were notable for WBC of 2.82 (differential 86% neutrophils, 10% lymphocytes), Hgb/HCT 10/29, Platelets of 14. Chemistry revealed BUN/Creatinine 28/3.1, and liver panel showed T bili 6.6 (direct 4.4, indirect 2.2), and ALT/AST 103/144 respectively. Initial antimicrobials included ceftriaxone and doxycycline. The patient had an increasing oxygen requirement, and subsequent CT chest revealed diffuse peribronchovascular nodular alveolar opacities with small bilateral pleural effusions. Abelcet was started at that time to include coverage for histoplasmosis. Intubation for hypoxic respiratory failure was soon required, and BAL from bronchoscopy showed progressively bloodier aliquots. Empiric Solumedrol at 125 mg every 6 hours was begun. Transbronchial biopsy revealed only intra-alveolar hemorrhage, with negative AFB and GMS stains. He slowly improved over the next few days, and was able to be extubated with return of normal kidney and liver function. After discharge from the hospital he returned to his normal state of health. Although initial leptospirosis serology was negative on admission, a repeat specimen sent to the CDC at hospital day 10 was positive, with a positive confirmatory microscopic agglutination test.

DISCUSSIONS:  Leptospirosis is caused by the spirochete Leptospira interrogans, which is distributed world-wide. The highest incidence occurs in tropical and sub-tropical regions. Animals, particularly rodents, can become colonized with Leptospira and shed the organism in urine which can contaminate soil or water leading to human infection. Clinical presentation includes the non-specific symptoms of fevers, headaches, and chills. Weil’s disease is the most severe manifestation of the disease, characterized by jaundice (with elevation of serum bilirubin elevated out of proportion to ALT/AST), acute renal failure, and thrombocytopenia. Pulmonary manifestations range from cough and dyspnea to hemoptysis. Imaging of the chest frequently reveals patchy infiltrates or consolidation, with occasional pleural effusions.

CONCLUSION:  Although infrequently encountered in the U.S., leptospirosis should be considered in patients with multi-organ dysfunction who have a travel history to endemic areas.

DISCLOSURE:  Paul Perry, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):31S. doi:10.1378/chest.136.4_MeetingAbstracts.31S-d
FREE TO VIEW

INTRODUCTION:  Juvenile Onset Recurrent Respiratory Papillomatosis (JORRP) is a rare condition characterized by recurrent growth of squamous papillomas in the upper and lower airways of children younger than twelve years old. It is caused by the cytopathogenic effect of human papilloma virus (HPV) 6, 11, 16/18 and 31/35/51, which is transmitted from active vaginal lesions of the infected mother at the time of birth. JORRP has a prevalence of 1.7–2.6/100,000 children. It typically presents with hoarseness which can progress to stridor. Multiple surgical interventions are often required to achieve local control of the disease and avoid airway obstruction. Involvement of the upper airway is common, especially the larynx. However, papillomas can also spread to the distal trachea and lung parenchyma in 5% of the cases. Several cases of malignant transformation have been reported in patients with JORRP in the setting of concomitant risk factors such as radiation or smoking. However, only few cases have been published describing malignant transformation of papillomas of the lung in the absence of these risks factors. We present a case of a 19 year-old woman with history of JORRP, who developed squamous cell carcinoma of the lung after being in clinical remission since the age of 5.

CASE PRESENTATION:  The patient was a 19 year-old female with history of JORRP who throughout her infancy required several laser interventions for treatment of papillomas of the upper airway. Her last intervention was at age four and her last normal bronchoscopy was at five years of age. She was a non-smoker and was never exposed to radiation. Throughout her adolescence she noticed occasional bronchitis symptoms that were treated symptomatically but no radiologic or invasive studies were performed. Ten month prior to admission she started to complain of frequent thoracic back and pleuritic chest pains. She developed chronic cough, dyspnea on exertion, and was treated with antibiotics for left lower lobe pneumonia. She eventually developed respiratory distress and was emergently intubated. On the day of admission a CT of the chest demonstrated complete collapse of the left lung, a 4.5 cm lower lobe mass and destruction of the fifth and sixth vertebral bodies (Figure 1). Bronchoscopy showed a 1 × 1 cm polyp in the left main stem bronchus, and a biopsy was obtained revealing dysplastic squamous epithelium. Subsequent pleural biopsy from Video-Assisted Thoracic Surgery showed a well differentiated squamous cell carcinoma and she was stratified as T4-N2-MO lung cancer (Figure 2).

DISCUSSIONS:  We present a case of a 19 year-old female with JORRP, who presented with squamous cell carcinoma of the lung in the absence of smoking or radiation. Prior reported cases of squamous cell carcinoma of the lung in adult patients with JORRP presented after many year of relapsing disease and frequent surgical interventions. In contrast, this patient presented after fourteen years of apparent clinical remission since the age of five. This case highlights that malignant transformation of papillomatous lesions of childhood JORRP is possible even after prolonged clinical remission.

CONCLUSION:  Malignant transformation of benign childhood papillomatosis can occur even in the presence of apparent clinical remission. Development of surveillance guidelines to guide long term follow-up of patients with JORRP is required to facilitate early diagnosis and treatment of malignant transformation.

DISCLOSURE:  Natalia Moguillansky, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):31S-e-32S. doi:10.1378/chest.136.4_MeetingAbstracts.31S-e
FREE TO VIEW

INTRODUCTION:  Dyspnea and pulmonary nodules are common reasons for pulmonary consultation. A thorough history and physical examination with basic supplemental testing can often significantly narrow your differential diagnosis and suggest more unusual diagnoses or secondary processes.

CASE PRESENTATION:  A seventy-two year old male presents to pulmonary clinic with two to three years of insidious onset dyspnea on exertion. He first noticed on subway stairs, but now with one flight of stairs or any running. He has also had six months of decreased appetite, early satiety, and 10lb weight loss. He denies fevers, chills, night sweats, chest pain, cough, hemotptysis, lower extremity edema, paroxysmal nocturnal dyspnea, orthopnea, change in vision, easy bruising, bleeding or epistaxis. He has a >60 pack year history, but quit one year ago, occasional alcohol and marijuana usage, no intravenous drug usage or blood transfusions. He was born in New York City and has lived there for all but one year spent living throughout Europe in 1965—Canary Islands, Spain, Italy, France, and Switzerland –as well as Arizona for his service time in the Army and Marine Air Reserves. He is a heterosexual male, divorced multiple times without children. He had worked as a film director, painter, and in art studios with rubber cement and thinner exposure, as well as volunteering at a correctional facility until 2001. His PPD converted in 2004, but was never treated. On exam, he was thin and pale, with normal vital signs and no exertional desaturation. His labwork demonstrated a normocytic anemia and a white blood cell count of 10,700 with a differential with 6% atypical lymphocytes, but a peripheral smear with rouleaux formation. He also had an ESR of 105mm/hr, and an elevated total protein/albumin ratio. His imaging demonstrated a right middle lobe nodule, a right upper lobe ground glass opacity (GGO), and diffuse emphysematous changes and micronodularity. His workup revealed a monoclonal Igm kappa spike with 10,850mg/dL. His serum viscosity was 3.8 and beta-2 microglobulin level 3.4. His peripheral blood, bone marrow, BAL and transbronchial biopsies all demonstrated monoclonal IgM kappa positive B-cell population consistent with Waldenstrom’s macroglobulinemia.He began treatment with dexamethasone, rituximab, and cyclophosphamide. After the third cycle his repeat CT imaging demonstrated resolution of micronodularity, and improvement in GGO and nodule. Additionally his IgM levels trended down, and his exercise tolerance returned to baseline.

DISCUSSIONS:  Waldenstrom’s macroglobulinemia was first described by Jan Waldenstrom in 1943, and demonstrates a lymphoplasmacytic B-cell lymphoma with intertrabecular bone marrow infiltration and an IgM monoclonal gammopathy. Pulmonary involvement has been reported to be present in 0–3% of all Waldenstrom’s cases, and its form is varied –masses, infiltrates, or effusions. Often pulmonary involvement is present at initial diagnosis, but it has also been reported as a later presentation. Symptoms often include dyspnea, though it has been hard to separate whether this is due to actual pulmonary involvement or anemia. Pathologic specimens have shown plasmacytoid lymphocyte infiltration, but notably there have also been reports of amyloid deposition. With the available data, pulmonary involvement does not seem to predict a worse prognosis.

CONCLUSION:  Waldenstrom’s macroglobulinemia is a relatively rare diagnosis, and pulmonary involvement is reported even less frequently, but it is important to know the potential manifestations in order to expeditiously and properly diagnose our patients. Additionally we need more data in order to better determine the prognosis and outcomes of this diagnosis.

DISCLOSURE:  Eric Bonura, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):32S. doi:10.1378/chest.136.4_MeetingAbstracts.32S-d
FREE TO VIEW

INTRODUCTION:  An extensive debate has existed over the association of sarcoidosis with cancer. In patients with certain malignancies like lymphomas, testicular cancer, lung cancer and melanomas an increased incidence of sarcoidosis has been classically reported (1). Only a single case report of coexistence of sarcoidosis with a gastric plasmacytoma has been described in the literature. The authors present, the second reported case of a plasmacytoma presenting concomitantly with sarcoidosis, and the first reported case occurring in a thoracic medullary plasmacytoma. The sarcoidosis resolved with the treatment of the plasmacytoma.

CASE PRESENTATION:  A 39 year-old previously healthy female presented for evaluation of pleuritic pain in the left lower chest. Chest radiography revealed a lytic lesion on the 9th rib, shown on computed tomography (CT) to be an irregular lytic mass suggestive of a plasmacytoma. Fine needle aspiration confirmed this diagnosis. Positron Emission Tomography (PET)/CT imaging again showed the hypermetabolic destructive lesion in the left ninth rib, with a standard uptake value (SUV) of 13. Hypermetabolic nodes were scattered in the mediastinum, hilum and inguinal region, with a maximum SUV of 6, along with ground glass opacities in both lung bases. The plasmacytoma was treated with local radiotherapy and dexamethasone. PET/CT scan of chest, abdomen and pelvis 6 months after treatment revealed improvement in the plasmacytoma and lymphadenopathy, as well as resolution of the ground glass opacities. PET/CT scan a year after treatment revealed recurrence of the hypermetabolic lymph nodes in the hilar, mediastinal, axillary and inguinal regions, with an SUV of 6.8 (increased from the initial value of 6), without worsening of the primary plasmacytoma. An inguinal lymph node biopsy was performed for evaluation of the lymphadenopathy. The biopsy showed necrotizing granulomas. Cultures for myobacteria and fungi were negative and the diagnosis of sarcoidosis was confirmed. The patient received consolidation and maintenance chemotherapy which resulted in further regression of the thoracic plasmacytoma as well as sarcoidosis. Imaging done after two years showed that the plasmacytoma remained unchanged and sarcoidosis lymphadenopathy had completely resolved.

DISCUSSIONS:  Plasmacytomas develop from the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin. They generally occur as a single lesion and are estimated to account for 5% of all plasma cell disorders. Although a few reported cases of sarcoidosis in association with multiple myeloma have been described, there has only been one previously described case of coexisting plasmacytoma and sarcoidosis in case of a gastric plasmacytoma (2 ). Sarcoidosis may represent the host immune response to malignancy. This could explain the higher prevalence of sarcoidosis with cancers. This may also explain why the sarcoidosis resolved with treatment for the tumor in our patient. Sarcoidosis can create a major diagnostic dilemma in cancer patients presenting with hypermetabolic lymph nodes. Biopsy proven disease must therefore always be sought prior to treatment.

CONCLUSION:  The first case of concomitant occurrence of sarcoidosis with a thoracic medullary plasmacytoma is reported. The possible association between sarcoidosis and cancer, and the implications of this association are discussed.

DISCLOSURE:  Toshita Kumar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):32S. doi:10.1378/chest.136.4_MeetingAbstracts.32S-e
FREE TO VIEW

INTRODUCTION:  Pulmonary blastoma is a rare tumor with histological similarity to fetal lung tissue. Comprising less than 0.5% of all malignant pulmonary neoplasms, approximately 200 case reports have been described in the literature. The classic biphasic pulmonary blastoma found in adults, consists of a malignant glandular component and a mesenchymal component that are both embryonal in appearance. This case report reviews the clinical, radiographic and pathologic findings in a 45 year old male diagnosed with classic biphasic pulmonary blastoma.

CASE PRESENTATION:  A 45 year old male with no previously diagnosed medical history presented to the clinic with complaints of shortness of breath, cough and exertional dyspnea. These symptoms occured over a 3 month time period, and he had never experienced these symptoms before. He described the cough as constant and non-productive, with no hemoptysis. He had not experienced fevers, weight loss, wheezing or chest pain during this time period. He is a current tobacco user and has a 40 pack year history of smoking. He has no known chemical or occupational exposures. A routine evaluation was initiated, which included basic blood laboratories and a chest radiograph. A mass was then discovered and he was referred for CT guided biopsy. Histologic examination revealed mesenchymal tissue and malignant glands with an embryonal appearance. Classic biphasic pulmonary blastoma was diagnosed based on pathology. He was subsequently referred to a cardiothoracic surgeon for resection.

DISCUSSIONS:  Pulmonary blastomas contain immature cellular components. The term blastoma has been used to describe tumors in both children and adults. Pulmonary blastoma has been subdivided into histologically characteristic groups: classic biphasic pulmonary blastoma, well-differentiated fetal adenocarcinoma and pleuropulmonary blastoma of childhood. Classic biphasic pulmonary blastoma contains malignant glands and mesenchymal tissue that are both embryonal in appearance. A well-differentiated fetal adenocarcinoma contains malignant glands and benign appearing mesenchymal tissue. A pleuropulmonary blastoma contains malignant glands of embryonal appearance and benign appearing epithelium. Patients with pulmonary blastoma can present asymptomatically with an incidental finding of a lung mass on chest radiography. Common symptoms include cough, hemoptysis, dyspnea, and chest pain. Symptoms such as fever, weight loss, and recurrent pneumonia may also present. Prognosis of pulmonary blastoma is poor, with a reported survival of 17% at 5 years. The primary treatment options include surgery and chemotherapy. Complete surgical resection in early presentation is regarded as the best treatment option. Surgical resection, however, is marred by large tumor size and frequent postoperative recurrence. In patients with inoperable disease, chemotherapy has been used with varying degrees of success. More studies are needed as no standardized chemotherapeutic regimen exists at this time, and it is unclear if radiotherapy is helpful.

CONCLUSION:  Pulmonary blastoma is a rare neoplasm affecting very few adults. Symptoms are nonspecific and most are incidentally discovered on routine radiography. There are no clearly defined treatment regimens, although complete surgical resection appears to confer the best prognosis. More trials are necessary to determine the most effective and appropriate therapies.

DISCLOSURE:  Jose Cantu, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):32S-f-33S. doi:10.1378/chest.136.4_MeetingAbstracts.32S-f
FREE TO VIEW

INTRODUCTION:  Primitive Neuroectodermal Tumors (PNET ) are rare thoracopulmonary malignancies grouped in the family of small round-cell tumors. There is overlap with the more common Ewing’s sarcoma which occur primarily in children and adolescents. PNET is exceedingly rare in adults with only a few published case reports. We report a case of PNET seen in an adult and discuss the radiographic and histopathological findings.

CASE PRESENTATION:  A 24 year old Armenian male presented with a one month history of cough, fevers, and left sided pleuritic chest pain. He was seen as an outpatient two weeks prior and did not respond to a course of antibiotics. His review of systems was negative and he otherwise had no significant past medical history. The patient was born in Armenia and came to the United States at the age of ten. He denied any prior tobacco use. At initial presentation he was febrile at 39.0 C, BP 120/64, HR 89, RR 20, and O2 sat 98% on RA. Chest auscultation revealed decreased breath sounds at the left base with dullness to percussion. There was a mild leukocytosis with chest roentgenogram revealing a left lower lobe infiltrate and an associated moderate sized pleural effusion. Thoracentesis with ultrasound guidance was attempted with no aspirate. A computed tomography of the chest showed a moderate left pleural effusion with significant heterogeneity and high attenuation; as well as possible nodularity, with nodularity extending along the mediastinal pleura. Transthoracic biopsy disclosed a malignant round-cell tumor positive for CD99 and vimentin. The microscopic features and results from the immunohistochemical studies pointed towards a diagnosis most consistent with PNET. The patient received a second opinion and ultimately had a Video-Assisted Thoracoscopic Surgery (VATS ) biopsy at an outside hospital which revealed the same diagnosis. He subsequently underwent neoadjuvant chemotherapy followed by surgical resection for local control.

DISCUSSIONS:  Primitive Neuroectodermal Tumors (PNET ) of the thoracopulmonary region are highly aggressive neoplasm’s with a mean survival of eight months. This tumor is seen predominantly in children and young adults and is thought to arise from embryonal cells migrating from the neural crest. Frequently this entity presents as a chest wall mass that may involve the pleura. The neoplasm is an undifferentiated small round-cell tumor which must be distinguished from neuroblastoma, rhabdomyosarcoma, and Ewing’s sarcoma. There is particular overlap with Ewing’s sarcoma which both share a reciprocal chromosomal translocation at (11;22). Positive staining with CD99 and neuron specific enolase with a negative stain for periodic acid Schiff differentiate it from other small round-cell tumors. Prognosis is very poor despite multimodal therapy with wide excision, chemotherapy, and radiotherapy; with a 2 year survival of 38% and a 6 year survival of 14%.

CONCLUSION:  Primitive Neuroectodermal Tumors (PNET ) of the chest wall are particularly aggressive tumors that should be considered in the differential diagnosis of chest wall masses regardless of age. Once diagnosed early wide excision along with chemotherapy and radiation must be undertaken for any hope of long term cure.

DISCLOSURE:  Hari Reddy, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):33S. doi:10.1378/chest.136.4_MeetingAbstracts.33S-c
FREE TO VIEW

INTRODUCTION:  Bronchus-associated lymphoid tissue lymphoma (BALToma) is a rare, low grade marginal zone lymphoma of the lung. Histologically, BALToma is characterized by the aggregation of extranodal, monoclonal B- lymphoid cells near the bronchial epithelium. The radiographic appearance of BALToma is variable, ranging from well circumscribed nodules, to dense consolidations with air bronchograms. We report two patients with histories of marginal zone lymphoma, who underwent resection of enlarging ground glass opacities (GGOs) found to be BALTomas.

CASE PRESENTATION:  Case 1A 69-year-old woman diagnosed with marginal zone lymphoma of the right lacrimal gland (CD 20+) was found to have multiple GGOs on her staging chest CT. The largest GGO was 2 cm in her left lower lobe. She was treated with radiation to the right eye and rituximab. Post treatment imaging demonstrated a stable left lower lobe GGO while the other GGOs decreased in size. Over the next 15 months the left lower lobe nodule increased in size (to 2.4cm) and density. She was referred for resection under the suspicion of bronchoalveolar carcinoma. As this lesion was deep within the parenchyma, a video-assisted thoracoscopic left lower lobectomy was performed. Histopathology confirmed a marginal zone B-cell lymphoma similar to the prior lacrimal gland lymphoma.Case 2A 55-year-old man with a history of a BALToma resected from his left upper lobe, was found after 5 years of serial imaging to have an enlarging 1.4cm GGO in the superior segment of his left lower lobe. The patient was referred for resection under the suspicion of bronchoalveolar carcinoma. He underwent a video-assisted thoracoscopic left lower lobe superior segmentectomy. Histopathology confirmed a marginal zone B-cell lymphoma (CD 20+, CD43+) similar to the prior left upper lobe BALToma.

DISCUSSIONS:  The radiographic appearance of BALToma can be variable. In a study of 24 patients the predominant patterns (88% of patients) were described as masses or mass-like consolidations most often multiple and with air bronchograms[1]. In a more recent study of 21 BALToma patients, the predominant CT scan pattern seen was consolidation with air bronchograms, followed by pulmonary nodules[2]. In eight patients GGOs were found (2 solitary, 6 multiple), but not comment was made towards stability over time.

CONCLUSION:  BALToma often poses a diagnostic dilemma due to its rarity, nonspecific presentation and diverse appearance on CT scan. It is important to consider BALToma in the differential of GGOs that are increasing in size, particularly in patients with a history of marginal zone lymphoma.

DISCLOSURE:  Kristina Bermas, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):33S-d-34S. doi:10.1378/chest.136.4_MeetingAbstracts.33S-d
FREE TO VIEW

INTRODUCTION:  Asian dust or yellow sand storms are seasonal meteorological phenomenon originating in the deserts of Mongolia and Northern China that affect much of East Asia. Exposure to ambient air particulate matter (PM) from dust storms has been associated with significant pulmonary morbidity and mortality. Desquamative Interstitial Pneumonia (DIP) is an idiopathic interstitial pneumonia that typically affects cigarette smokers. We present a unique case of a nonsmoker who developed DIP necessitating lung transplantation after exposure to an Asian dust storm event in South Korea.

CASE PRESENTATION:  A 47 year old male lifetime nonsmoker was emergently referred for lung transplant evaluation for progressive hypoxemia due to pulmonary fibrosis. Patient was in his usual state of health until he was exposed to yellow dust storm while working in South Korea one year before presentation. He developed acute shortness of breath and cough immediately after the dust storm which never completely resolved. Subsequent workup including CT chest showed diffuse ground glass infiltrates. Bronchoscopy was macroscopically normal with a lymphocyte predominant lavage with no transbronchial biopsies preformed. He was empirically treated with systemic steroids with no improvement in his symptoms. Patient continued to worsen both subjectively and objectively and underwent a surgical lung biopsy which showed DIP type pattern. Following the surgical lung biopsy, patient began to have worsening hypoxemia leading to prolonged hospitalization and transfer to our institution for emergent lung transplant evaluation. His clinical condition worsened after developing an iatrogenic pneumothorax and he emergently underwent bilateral lung transplantation on ECMO support as a bridge to transplant due to his rapid deterioration. The explanted lung pathology demonstrated an abundance of alveolar macrophages within the alveolar spaces and many of the macrophages appeared to contain some pigment. Polirization of multiple tissue blocks showed minimal birefringent material. The pathology was felt to be consistent with an exposure related injury with DIP pattern.

DISCUSSIONS:  DIP, typically seen in male smokers who present in their 3rd or 4th decade of life, is considered to be a non-specific pulmonary reaction to injury, and is pathologically characterized by the presence of numerous macrophages within the airspace with minimal fibrosis. DIP is known to occur in non-smokers most commonly in the context of pneumoconiosis, drug reactions or inborn errors of metabolism. Asian or yellow dust storm, a weather phenomenon originating in the desert regions of China and Mongolia, when combined with air pollutants from human activities have resulted in increased pulmonary morbidity and mortality mainly in patients with underlying lung disease (COPD or asthma). In animal studies, exposure to concentrated PM from an actual Asian dust storm has been shown to cause lung inflammation and injury associated with elevated IL-6 in the bronchoalveolar lavage fluid. There is a paucity of knowledge in the English literature regarding the association of interstitial lung disease with exposure to Asian dust storm but scant evidence does exist in the Chinese literature. Our patient had no known lung disease and had an active life style prior to his exposure to the PM from the Asian dust storm in 2007. He developed acute respiratory symptoms after the exposure with CT imaging indicating indicative of an inflammatory reaction which progressed on a relentless course necessitating bilateral lung transplantation. This is the only reported case of bilateral lung transplant for Asian dust exposure in the literature with the histopathology of the explanted lungs being consistent with a DIP type reaction to an exposure related injury.

CONCLUSION:  In susceptible patients, exposure to particulate matter from Asian dust storm may be associated with DIP type pattern of lung injury.

DISCLOSURE:  Ravindra Gudavalli, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):34S. doi:10.1378/chest.136.4_MeetingAbstracts.34S-c
FREE TO VIEW

INTRODUCTION:  The September 11, 2001 terrorist attack in New York City (NYC) that caused the collapse of the World Trade Center (WTC) resulted in the release of large amounts of smoke, debris, and particulate matter into the air. Many people involved in the initial rescue and recovery effort were exposed to these respirable materials. There has been an increasing number of pulmonary diseases reported in association with this exposure. We report a case of Swyer-James-Macleod Syndrome (SJMS) in a WTC first responder.

CASE PRESENTATION:  A 38-year-old male nonsmoker presented with 2 years of dyspnea on minimal exertion, cough, and wheezing. He was a first responder at WTC, “Ground Zero,” during which time he was exposed to particulate matter in the air. Prior to this, he was able to run miles and perform various exercises for his police department training. In 2002, he underwent treatment for “pneumonia”. In 2007, during WTC screening at Mt. Sinai, NYC, he reported a negative chest radiograph. Review of systems revealed occasional right-sided chest pressure on exertion. He noted symptomatic relief with albuterol meter-dose-inhaler. Physical examination showed reduced air-entry over the right chest without wheezing or rhonchi. Oxygen saturation on room air decreased to 92% while walking. Pulmonary function testing showed mild restrictive lung disease with minimal response to bronchodilator therapy. Computed tomography (CT) of chest demonstrated a hyperlucent right upper lobe with oligemic lung field consistent with SJMS (Figure 1).

DISCUSSIONS:  To our knowledge, this is the first case reported of SJMS occuring after exposure to WTC respirable particulate matter. Exposure to materials in the air caused by the collapse of the WTC has been reported to result in bronchial responsiveness, cough, sarcoid-like granulomatous disease as well as bronchiolitis obliterans[2]. SJMS is a rare disorder characterized radiographically by a unilateral hyperlucent lung. It is believed to be a postinfectious manifestation of childhood bronchiolitis obliterans caused by injury of the immature lung. Acute viral respiratory infection during the first 8 years of life is thought to be the main causative factor, however about 40% of cases have documented no history of episodes of childhood respiratory infection[1]. The diagnosis is usually made based on clinical and radiographic findings rather than by pathology. When pathologic specimens have been examined, they have shown bronchiolitis obliterans with various degrees of chronic inflammation, fibrosis, and dilatation of airways and air spaces distal to the obstructed bronchioles. Air trapping results from air entering the spaces via collateral air drift but being unable to exit due to bronchiolar obstruction[2]. Chest CT Scan is valuable because it eliminates other diagnoses that may mimic unilateral hyperlucency, such as central bronchial obstruction, cysts, and vascular disease[1]. The prognosis and severity of SJMS is variable and depends on the development of bronchiectasis and frequency of recurrent pneumonia[1].

CONCLUSION:  Although pulmonary disease has been reported following WTC exposure, this is the first reported case of SJMS. Our previously healthy male presented with dyspnea on exertion, persistent cough, wheezing, and chest pressure following exposure to WTC debris. We believe the inhalation of toxic material during the intense short-term exposure to WTC led to bronchiolitis obliterans and subsequently to SJMS. Absence of respiratory symptoms with normal functioning capacity prior to 9/11/2001 emphasizes that the WTC exposure was the cause of his disease. This suggests that when presented with a case of SJMS, a history of toxic inhalation should be elicited along with that of recurrent respiratory infections.

DISCLOSURE:  Ruby Varghese, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2009;136(4_MeetingAbstracts):34S-d-35S. doi:10.1378/chest.136.4_MeetingAbstracts.34S-d
FREE TO VIEW

INTRODUCTION:  Sirolimus (SRL) is a potent immunosuppressant drug being used with increasing frequency in solid organ transplant patients. Pulmonary toxicity secondary to SRL has been shown to cause interstitial pneumonitis, BOOP, alveolar hemorrhage and noncardiogenic pulmonary edema. Pulmonary alveolar proteinosis (PAP) is a rare pulmonary toxicity of SRL. There are only three reported cases, two in lung and one in renal transplant patient, of PAP secondary to SRL. We present a case of PAP in a 54 yr old man with cadaveric renal transplant which was treated by discontinuing Sirolimus.

CASE PRESENTATION:  A 54 year-old male with a history of cadaveric renal transplant presented with 1 month history of increasing dyspnea, 4 pillow orthopnea and dry cough. His medications included mycophenolate, SRL, prednisone and Bactrim. A month prior to his admission he was treated for presumed pneumonia with multiple courses of antibiotics including doxycycline and moxifloxacin. His history was significant for ESRD secondary to PCKD and a 45 pack year history of tobacco use. On examination he was afebrile and dyspenic requiring 12L high flow nasal cannula. Lung examination revealed crackles throughout. Labs included a WBC count of 8.2 with 79% granulocytes. ABG was 7.43/28/61/91% on 60 % FiO2. A CXR showed diffuse patchy infiltrates bilaterally. A CT of the chest with contrast demonstrated diffuse ground glass opacities with interlobular septal thickening (crazy paving pattern). He was started on Zosyn, vancomycin and azithromycin. A TEE showed an EF of 65% with diastolic dysfunction. Bronchoalvoelar lavage was positive for milky fluid which was hypocellular and contained rare vacuolated alveolar macrophages that stained positive for PAS. He underwent video assisted thoracoscopic surgery (VATS) with right upper and lower lobe wedge resection. Pathology revealed pulmonary alveolar proteinosis. SRL was discontinued. All cultures were negative for infection hence antibiotics were also discontinued. Respiratory status improved and he was discharged home on supplemental oxygen. At follow-up two months later he only required supplemental oxygen with activity and CXR showed significant improvement.

DISCUSSIONS:  Pulmonary Alveolar Proteinosis (PAP) is a rare disorder characterized by accumulation of PAS-positive phospholipoproteinaceous material within alveoli with minimal interstitial inflammation or fibrosis. It has a variable clinical presentation and course. Most cases are acquired but it can also be congenital or secondary. The secondary causes include inhalation syndromes immunodeficiency disorders and hematopoietic disorders. Patients usually present with non-productive cough, dyspnea on exertion and low grade fever. Physical examinations often reveals inspiratory crackles and clubbing in up to one third of cases. Patients with advanced disease may have central and peripheral cyanosis. Pulmonary function test show a restrictive ventilatory defect. CXR finding can range from perihilar (bat’s wing pattern) to predominantly peripheral or basal consolidation. High resolution computed tomographic (HRCT) scan show widespread consolidation with thickened interlobular septa producing the so called “crazy paving” pattern. Diagnosis can be made with confidence on the basis of the appearance of HRCT scan of images in conjunction with alveolar lavage. Tissue pathology is another modality that can be used for a more definitive diagnosis. The treatment depends on the distinct class of PAP. Our patient was successfully treated with discontinuation of the offending agent with marked improvement in respiratory status.

CONCLUSION:  Pulmonary alveolar proteinosis can present as a complication of Sirolimus. Effort should be made to rule out infections but early recognition of this association may avoid invasive investigations. Discontinuation of SRL may lead to complete resolution of PAP.

DISCLOSURE:  Kapil Dhawan, No Financial Disclosure Information; No Product/Research Disclosure Information

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543