Sharma Aman, Gopalakrishan Dharmesh, Nada Ritambhra, Kumar Susheel, Dogra Sunil, Aggarwal Mayank M, Gupta Rajesh, Minz Ranjana W, Kakkar Nandita, Vashishtha Rakesh K, Singh Surjit
Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Int J Rheum Dis. 2014 Jun;17(5):562-72. doi: 10.1111/1756-185X.12223. Epub 2013 Nov 14.
Systemic vasculitides are great masqueraders and at times their presenting manifestations can be very different from the usual recognized patterns. Such uncommon presentations of granulomatosis with polyangiitis (Wegener's granulomatosis), classical polyarteritis nodosa and unclassifiable vasculitides are described here with the relevant review of literature.
All patients diagnosed as having systemic vasculitides and classified as having granulomatosis with polyangiitis (Wegener's granulomatosis), classic polyarteritis nodosa, microscopic polyangiitis and unclassifiable vasculitis according to EMEA consensus methodology and followed up prospectively from June 2007 to December, 2011 were included. Details of uncommon presentations of these disorders were identified.
Seventy-nine patients with systemic vasculitides were seen under our rheumatology services during this period. These included 45 patients with granulomatosis with polyangiitis (Wegener's granulomatosis), 18 with classic polyarteritis nodosa, five with microscopic polyangiitis, four with Churg-Strauss syndrome and seven with unclassifiable vasculitis. The uncommon presentations of granulomatosis with polyangiitis were a tumefactive subcutaneous mass in the thigh; prostatomegaly with obstructive uropathy and advanced renal failure; and predominant gastrointestinal (GI) vasculitis with thrombocytopenia and coagulopathy at presentation. The uncommon manifestations of classic polyarteritis nodosa were secondary antiphospholipid antibody syndrome and Budd-Chiari syndrome. One patient with massive lower GI bleeding required surgical resection of the large bowel which showed isolated necrotizing granulomatous GI vasculitis. Single organ vasculitis of the GI tract was diagnosed.
Systemic necrotizing vasculitides may present with uncommon manifestations and a high index of suspicion is required for early diagnosis and prompt treatment to prevent adverse outcomes.
系统性血管炎极具伪装性，有时其呈现的表现与常见的典型模式大不相同。本文描述了肉芽肿性多血管炎（韦格纳肉芽肿）、经典结节性多动脉炎及无法分类的血管炎的此类罕见表现，并对相关文献进行综述。
纳入所有根据欧洲药品管理局（EMEA）共识方法诊断为系统性血管炎，且归类为肉芽肿性多血管炎（韦格纳肉芽肿）、经典结节性多动脉炎、显微镜下多血管炎及无法分类的血管炎，并于2007年6月至2011年12月进行前瞻性随访的患者。确定这些疾病罕见表现的详细情况。
在此期间，我们的风湿病科共诊治了79例系统性血管炎患者。其中包括45例肉芽肿性多血管炎（韦格纳肉芽肿）患者、18例经典结节性多动脉炎患者、5例显微镜下多血管炎患者、4例变应性肉芽肿性血管炎患者及7例无法分类的血管炎患者。肉芽肿性多血管炎的罕见表现为大腿部肿胀性皮下肿块；前列腺肿大伴梗阻性尿路病及晚期肾衰竭；以及以胃肠道（GI）血管炎为主，伴血小板减少和凝血障碍。经典结节性多动脉炎的罕见表现为继发性抗磷脂抗体综合征和布加综合征。1例下消化道大出血患者需要手术切除大肠，术中显示孤立性坏死性肉芽肿性胃肠道血管炎，诊断为胃肠道单器官血管炎。
系统性坏死性血管炎可能以罕见表现出现，早期诊断和及时治疗需要高度怀疑，以防止不良后果。
