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Clin Rheumatol ; 26(9): 1537-9, 2007 Sep.
Article in English | MEDLINE | ID: mdl-17047893

ABSTRACT

Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms. Her clinical picture was consistent with BD. She was initially treated with prednisone and cyclophosphamide, followed by maintenance therapy with infliximab. This case describes a unique patient with OCA who developed BD with large pulmonary aneurysms and has remained stable for over 1 year while receiving infliximab.


Subject(s)
Albinism, Oculocutaneous/complications , Aneurysm/etiology , Behcet Syndrome/complications , Heart Diseases/etiology , Thrombosis/etiology , Adolescent , Black or African American , Antibodies, Monoclonal/therapeutic use , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Female , Humans , Infliximab , Pulmonary Artery/physiopathology , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Vasculitis/drug therapy , Vasculitis/etiology
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