Carels T, Verbeken E, Blockmans D
Department of Internal Medicine, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.
Clin Rheumatol. 2005 Feb;24(1):83-6. doi: 10.1007/s10067-004-0998-0. Epub 2004 Nov 24.
We describe a 63-year-old man who presented with an inflammatory aortic aneurysm. The patient had p-ANCA antibodies directed against myeloperoxidase. A diagnosis of idiopathic periaortitis was made. Seven years later, he was rehospitalized because of fever, weight loss, and polyneuropathy. After revision of the aortic biopsy, which showed necrotizing vasculitis with palisading granuloma, a diagnosis of Wegener's granulomatosis (WG) was made. This case report illustrates an unusual disease course in WG, resembling large vessel vasculitis, and we discuss the possible mechanisms of large vessel involvement in this form of vasculitis.
我们描述了一名63岁患有炎性主动脉瘤的男性患者。该患者存在针对髓过氧化物酶的抗蛋白酶3-抗中性粒细胞胞浆抗体（p-ANCA）。诊断为特发性主动脉周炎。七年后，他因发热、体重减轻和多发性神经病再次住院。再次检查主动脉活检显示为伴有栅栏状肉芽肿的坏死性血管炎，诊断为韦格纳肉芽肿（WG）。本病例报告说明了WG中一种不寻常的病程，类似于大血管血管炎，并且我们讨论了这种血管炎形式中出现大血管受累的可能机制。
