Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.
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.
Endo
Lois M
LM
Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA.
Rowe
Steven M
SM
Romp
Robb L
RL
Buckmaster
Mark A
MA
Atkinson
T Prescott
TP
D000293:Adolescent; D001741:Black or African American; D016115:Albinism, Oculocutaneous / Q000150:complications*; D000783:Aneurysm / Q000209:etiology*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D006331:Heart Diseases / Q000209:etiology*; D006801:Humans; D000069285:Infliximab; D011651:Pulmonary Artery / Q000503:physiopathology; D013927:Thrombosis / Q000209:etiology*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology
D000911:Antibodies, Monoclonal; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab