SESSION TITLE: Miscellaneous Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: The Churg-Strauss syndrome (CSS) is a rare necrotizing vasculitis that often affects the lungs as a result of the assault of small and medium caliber vessels forming eosinophilic granulomas. We describe the case of a woman with erroneous treatment for tuberculosis, in which CSS symptoms presented during pregnancy and intensified after delivery.
CASE PRESENTATION: Female, 32 years old, mulatto, brazilian. Came to our service reporting no improvement to empirical treatment for tuberculosis. Related a 10 months earlier history of dry cough, nasal congestion, rhinorrhea and dyspnea. She was 4 months pregnant and didnt realized radiography, being prescribed Amoxicillin. With no clinical improvement, went to an allergist who prescribed a new course of antibiotics and prednisone which she didnt use.The symptoms progressed with worsening cough, dyspnea, mucoid sputum, and low fever. Performed allergy tests,24 hours pH measurement, which were all negative. Searched for pulmonologist who prescribed acetyl cefuroxime, oral corticosteroid and nasal budesonide. Again did not use corticosteroids. The labor progressed uneventfully to term. Approximately 15 days after delivery, respiratory symptoms were severe, clinical worsening occurred with high fever, delirium, episodes of pleuritic pain and hemoptysis. Evaluated by another pulmonologist who requested a chest radiography showing bilateral interstitial infiltrate. Submitted to two tuberculin tests and 3 sputum tests, all negative and despites that, tuberculostatic scheme was started empirically. Worsening after tuberculosis medication was evident and was admitted to the ICU in bad clinical general condition, with hallucinations, decreased breath sounds and bibasilar crackles.Therapy for tuberculosis was suspended. Chest CT scan showed bilateral alveolar infiltrates and chronic sinusitis on facial CT scan. Epigastric pain, hypertension, paresthesia on left hand, a six pound lost, arthritis appeared on the following days. Blood tests showed: Hemoglobin 10mg/dl, leukocytosis (26.000), peripheral eosinophilia of 15%.Parenteral antibiotics was started. Rheumatology Panel: positive c-ANCA. After clinical stabilization, she underwent surgical open lung biopsy. Results: necrotizing eosinophilic granulomas in the surrounding vessels and alveoli area. After the surgical procedure hydropneumothorax appeared and was drained. It complicated with thoracic empyema. She underwent surgical lung decortication and antimicrobial treatment evolving favorably. Based on the clinical presentation and tests she was diagnosed with CSS. After a methylprednisolone pulse therapy and cyclophosphamide presented with significant clinical and laboratory improvement. Pulmonary function tests displays restriction pattern with moderated reduction of carbon monoxide diffusion. Chest CT revealed complete resolution of pulmonary infiltrates. Today she is asymptomatic.
DISCUSSION: Patients with CSS benefits of early detection, accurate diagnosis, aggressive treatment and periodic monitoring. The diagnosis of CSS needs 4 of 6 criteria: asthma(which she never had making the diagnosis difficult), peripheral eosinophilia above 10%, sinusitis, pulmonary infiltrates, vasculitis with extravascular eosinophil and mononeuropathy or polyneuropathy. Treatment should be based on the aggressiveness of the symptom and patients should receive systemic corticosteroids and a immunosuppressant such as cyclophosphamide. The drop in peripheral of eosinophil count is directly proportional to the effectiveness of treatment.
CONCLUSIONS: CSS is a rare disease, peculiarly in pregnant women, that needs an accurate and fast diagnosis. The presence of a pregnancy status implies an immune maternal-fetal tolerance aiming the viability of the fetus and for this reason the patient's symptoms were mild during pregnancy and intensified after childbirth.
Reference #1: Churg-Strauss syndrome associated with pregnancy.Hiyama J, Shiota Y, Marukawa M, Horita N, Kanehisa Y, Ono T, Mashiba H. Department of Internal Medicine, Kure Kyosai Hospital.Internal Medicine (Tokyo, Japan) [2000, 39(11):985-990]
Reference #2: Churg-Strauss syndrome. Pagnoux, Christian; Guilpain, Philippe; Guillevin, Loïc. Current Opinion in Rheumatology:January 2007 - Volume 19 - Issue 1 - p 25-32
Reference #3: J Clin Rheumatol. 2002 Jun;8(3):151-6.Churg-Strauss syndrome and pregnancy: successful treatment with intravenous immunoglobulin.Rutberg SA, Ward DE, Roth BJ.Department of Internal Medicine, Madigan Army Medical Center, Tacoma, WA 98431, USA.
DISCLOSURE: The following authors have nothing to disclose: Gilmar Zonzin, Christian Boechat, Gabriela Souza
No Product/Research Disclosure Information