Vivante Asaf, Bujanover Yoram, Jacobson Jeffrey, Padeh Shai, Berkun Yackov
Department of Pediatrics, Lily and Edmond Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel.
Rheumatol Int. 2009 Mar;29(5):575-7. doi: 10.1007/s00296-008-0730-5. Epub 2008 Oct 11.
Behçet disease (BD) is an inflammatory disorder of unknown origin. We present here an unusual case of juvenile Behçet with hemoptysis due to large pulmonary artery aneurysms (PAA), large intra-cardiac thrombus and prolonged fever, which posed several therapeutic challenges. In this case, a 14-year-old boy was admitted with a 3-month history of fever, painful oral ulcers, skin rash and intermittent hemoptysis. A high resolution helical computed tomography angiogram demonstrated thrombi in the right ventricle, two large aneurysms located in the right lung and two smaller ones in the left. The patient was successfully treated with colchicine, prednisone, cyclophosphamide and enoxaparine. A discussion about PAA and intracardiac thrombi and their role in BD is provided in this case.
白塞病（BD）是一种病因不明的炎症性疾病。我们在此呈现一例不寻常的青少年白塞病病例，该患者因巨大肺动脉瘤（PAA）、巨大心内血栓及长期发热而咯血，这带来了诸多治疗挑战。在此病例中，一名14岁男孩因发热、疼痛性口腔溃疡、皮疹及间歇性咯血3个月入院。高分辨率螺旋计算机断层血管造影显示右心室内有血栓，右肺有两个大动脉瘤，左肺有两个较小的动脉瘤。该患者通过秋水仙碱、泼尼松、环磷酰胺和依诺肝素成功治愈。本病例对白塞病中肺动脉瘤和心内血栓及其作用进行了讨论。
