抗中性粒细胞胞质抗体相关性小血管炎的神经系统损害

Clinical analysis of nervous system involvement in ANCA associated vasculitis

目的总结显微镜下多血管炎（MPA），韦格纳肉芽肿病（WG）和Churg—Strauss综合征（CSS）患者的神经系统损害特点。方法回顾性分析1991-2006年住院治疗的104例抗中性粒细胞胞质抗体（ANCA）相关性血管炎患者，其中MPA58例，WG31例，CSS15例。总结患者的临床特征和神经系统损害特点。结果患者诊断时平均年龄：MPA为50．7岁，WG为47．5岁，CSS为41．7岁。MPA组和WG组男女比例相似．但CSS组男性多见。MPA、WG和CSS组中分别有25例（43％），16例（52％）和13例（87％）出现周同或中枢神经损害．三组均以周围神经损害多见，但WG的颅神经受累明显高于其他两组。合并神经损害和无神经损害的患者血管炎活动指数（BVAC）在三组差异均无统计学意义。所有患者均进行了ANCA检测，MPA组中44例（16％）阳性，WG组中24例（77％）阳性，CSS组中4例（27％）阳性。结论神经系统损害是ANCA相关性血管炎最常见的临床表现之一，风湿病医生应重视神经损害的诊断和及时治疗。

Objective To describe the clinical features of nervous system involvement in patients of ANCA associated systemic vasculitis, including microscopic polyangiitis （MPA）, Wegener＇s granulomatosis （WG） and Churg-Strauss Syndrome （CSS）. Methods We retrospectively reviewed medical records of 104 patients （58 MPA, 31 WG and 15 CSS） during 1991 to 2006 of PUMC hospital. The diagnose were made based on the American College of Rheumatology and Chapel Hill criteria. Nervous system manifestations were the focus of this study. Results The average age （years） at diagnosis for MPA was 50.7, WG was 47.5 and CSS was 41.7. Gender distribution was similar in both MPA and WG groups, except CSS, in which the majority of patients were males. Twenty-five （43%） MPA, 16 （52%） WG and 13 （87%） CSS patients suffered from nervous system damage. The clinical features of the three groups were similar. The prevalence of peripheral neuropathy was much higher than central nervous involvement. However, cerebral nerve involvement was more prominent in WG than in MPA and CSS. There was no statistical significant difference in Berminham vasculitis activity score （BVAC） between those who had nervous system involvement and those without in the three groups. ANCA was positive in 44（76%） MPA patients, 24 （77%） WG patients and 4（27%） CSS patients. Conclusion Nervous system involvement is one of the most common manifestations in ANCA associated vasculitis. Rheumatologist should pay attention to its clinical features and give proper treatments.