SESSION TITLE: Interstitial Lung Disease Case Report Posters II
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Behcet’s disease is an idiopathic inflammatory disorder characterized by recurrent episodes of oral ulcerations and associated manifestations in a variety of other organ systems. Pulmonary disease has a prevalence between 1 and 7.7%. Pulmonary vascular abnormalities are most common. Parenchymal disease with pathologic correlation has been infrequently described, particularly in children.
CASE PRESENTATION: 10 y/o male first evaluated by rheumatology for 2 years of recurrent oral ulcers, rash, joint and abdominal pain. He was diagnosed with Behcet's disease. Colchicine was initiated with improvement in ulcerations and rash. He reported dyspnea on exertion and cough, which prompted referral to pulmonary. He described feeling “decreased lung capacity” with minimal activity. Cough was worse with illnesses and activity. Asthma therapies had been trialed without improvement in symptoms or lung function. Physical exam was remarkable for multiple healing skin lesions. Exam was otherwise normal with normal growth parameters and vital signs. Chest CT showed diffuse ground glass and consolidative opacities. FEV1/FVC: 89, FEV1: 59%, FVC: 58%, TLC 58% and adjusted DLCO 43%. After two weeks of Colchicine, DLCO normalized to 101%, while other parameters were unchanged. Lung biopsy showed multiple well circumscribed foci of alveolar spaces filled with neutrophils and macrophages. Iron stain showed moderate interstitial and intra-alveolar hemosiderin laden-macrophages without vasculitis. Bronchoscopy showed patchy mucosal erythema and 5 small, shallow base ulcerations in the right middle lobe. BAL had 64% neutrophils, 4% lymphocytes, 28% macrophages, 4% eosinophils, few hemosiderin-laden macrophages and no organisms. There was no growth on culture. Prednisone 30 mg BID (2 mg/kg/day) was initiated for 2 weeks followed by 30 mg daily for one month. Azathioprine was started at 50 mg once daily. (1.4 mg/kg/day) Prednisone was slowly tapered every 4 weeks. FVC improved to 73% initially and stabilized between 68-70% during steroid taper. Chest CT following 2 months of treatment showed improved ground glass opacities. Cough and dyspnea have improved.
DISCUSSION: Ground glass opacities have been described in adults with Behcet's disease. Hemosiderin-laden macrophages on this patient's lung biopsy suggest multiple small areas of hemorrhage. Neutrophilic infiltrate gave the appearance of an acute pneumonia, however he had clinical and radiologic improvement with immunosuppressive medications and was not treated with antibiotics.
CONCLUSIONS: Behcet's disease is a rare disorder, particularly in children. Pulmonary parenchymal disease does occur in children and can improve with immunosuppressive therapy.
Reference #1: Erkan F, Gul A, Tasali E. Pulmonary Manifestations of Behcet's Disease. Thorax. 2001; 56:572-578.
Reference #2: Seyahi E, Melikoglu M, Akman C, et al. Pulmonary Artery Involvement and Associated Lung disease in Behcet Disease. Medicine. 2012;91(1):35-48.
DISCLOSURE: The following authors have nothing to disclose: Michelle Ratkiewicz
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