Endo Lois M, Rowe Steven M, Romp Robb L, Buckmaster Mark A, Atkinson T Prescott
Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA.
Clin Rheumatol. 2007 Sep;26(9):1537-9. doi: 10.1007/s10067-006-0426-8. Epub 2006 Sep 19.
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.
白塞病(BD)是一种可能累及多种器官的系统性血管炎。我们描述了一名患有眼皮肤白化病(OCA)的女孩,在对其腘动脉闭塞进行初步评估时,发现有多个心脏血栓。她后来出现咯血,这是双侧肺动脉瘤的结果。她的临床表现符合白塞病。她最初接受泼尼松和环磷酰胺治疗,随后用英夫利昔单抗进行维持治疗。本病例描述了一名患有OCA的独特患者,其患白塞病并伴有大型肺动脉瘤,在接受英夫利昔单抗治疗期间已保持稳定超过1年。