INTRODUCTION:Pulmonary capillary hemangiomatosis (PCH) is a rare locally invasive vascular neoplasm of the lung characterized by the presence of numerous thin-walled capillary-sized blood vessels proliferating diffusely throughout the pulmonary interstitium, pulmonary vasculature and airways. PCH typically occurs in young patients who have signs and symptoms of pulmonary hypertension. We present a case of PCH without pulmonary hypertension and without the usual radiological features associated with this disorder.
CASE PRESENTATION:A 41-year-old lady presented with non-productive cough and dyspnea on exertion of six months duration. She denied fever, chills, chest pain, and hemoptysis. She has history of stable discoid lupus and hypertension that is controlled with HCTZ and amlodipine. Four years ago, the patient had a febrile illness with cough and was diagnosed with pneumonia. Her symptoms resolved after treatment with antibiotics. Two years ago, her cough recurred and was associated with one episode of blood-tinged sputum. She was hospitalized. A computed tomographic (CT) scan of the chest demonstrated bilateral upper lobe ground glass opacities without significant mediastinal or hilar adenopathy. She received intravenous antibiotics and was subsequently lost to follow-up. She is a non-smoker, works as a counselor at a school and is married with two healthy children. Physical examination, blood counts, liver and renal functions and urinalysis were all normal. The arterial blood gas on room air at rest demonstrated a PaO2 of 68 mm Hg and PaCO2 of 34 mmHg. Extensive serological evaluation failed to find any indication of angiitis, collagen vascular, autoimmune disease, or HIV infection. The electrocardiogram and echocardiogram were both normal, without any findings suggestive of pulmonary hypertension. Chest radiography revealed patchy ground glass opacities mainly in both upper lung zones. A recent CT scan of the chest demonstrated multiple ground glass nodules with patchy infiltrates in the right and left lung zones and sub centimeter prevascular, pretracheal and subcarinal lymph nodes. High-resolution CT (HRCT) images showed that each lesion consisted of three zones fairly well demarcated, a central focal nodular opacity, surrounding ground glass opacities, and some demonstrating a dense periphery - reverse halo sign (figure 1). Due to the persistent nature of the infiltrates and the presence of symptoms, the patient underwent video assisted thoracoscopic surgery. Pathology demonstrated thin wall capillary proliferation extending into the peribronchial and perivascular interstitium and the lung parenchyma (figure 2). Immunopathological stains in conjunction with the microscopic findings were consistent with PCH.
DISCUSSIONS:Pulmonary capillary hemangiomatosis is an extremely rare disorder characterized by the proliferation of multiple layers of capillaries that expand alveolar septae, and often invade bronchial walls and pleura. It commonly is misdiagnosed as pulmonary veno-occlusive disease, and the correct diagnosis usually is not made until autopsy. Presentations include dyspnea and hemoptysis. Pulmonary hypertension is a hallmark of PCH, which was not present in our patient. The most commonly reported HRCT findings of smooth interlobular septal and intralobular interstitial thickening with peribronchovascular thickening were absent in our patient. She had multiple ground glass nodules with patchy infiltrates in both lungs. The dysregulated angiogenesis seen in PCH patients possibly may result from either the neoplastic proliferation of capillaries or the sequelae of a prior pulmonary infection. Based on a previous case report of successful treatment with doxycycline, our patient was placed on doxycycline. Her symptoms have improved over six months and the follow up CT scan had no progression.
CONCLUSION:The absence of pulmonary hypertension does not exclude the diagnosis of PCH. PCH should be considered in the differential diagnosis of patients with non-resolving infiltrates, or ground glass subcentimeter nodules.
DISCLOSURE:Arun Devakonda, No Financial Disclosure Information; No Product/Research Disclosure Information