Resolution of Behcet's Syndrome Associated Pulmonary Arterial Aneurysms with Infliximab

Introduction: We describe the successful treatment of pulmonary arterial aneurysms in Behcet's syndrome using a tumor necrosis factor (TNF) inhibitor. Methods: A case is reported of Behcet's syndrome complicated by pulmonary arterial aneurysms that responded to anti-TNF therapy. This is accompanied by a literature review of previously published cases. We searched the English language medical literature using the PubMed and Medline search terms: "Behcet's," "Pulmonary aneurysms," and "infliximab," "etanercept," or "adalimumab." Results: A 43-year-old man with a 6-month history of oral and genital ulcers, weight loss, and fatigue developed arterial aneurysms in the common carotid and common iliac arteries and thromboses in a femoral vein and pulmonary arteries. Treatment with high-dose oral corticosteroids and pulsed intravenous cyclophosphamide was initiated but while on treatment he developed pulmonary arterial aneurysms with hemoptysis. His treatment was changed to intravenous infliximab with methotrexate to which he showed a good response with marked clinical improvement, reduction in his inflammatory markers, and regression of the pulmonary arterial aneurysms. The review of the literature identified 3 reported cases of treatment of pulmonary arterial aneurysms in Behcet's syndrome with anti-TNF therapy, with good outcomes in each case. Conclusions: Pulmonary artery aneurysms are important complications of Behcet's syndrome. Anti-TNF inhibitors should be considered in patients who do not respond to treatment with corticosteroids and cyclophosphamide. (C) 2011 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 41:482-487