Objective To improve the awareness and the methods of diagnosis about the clinical characteristics of primary small vessel vasculitis.Methods Clinical data from 52 patients with primary small vessel vasculitis were collected and their symptom, pulmonary image, antineutrophil cytoplasmic antibody(ANCA) results and pathological features were further analyzed.Results Among 52 cases,33 cases were microscopic polyarteritis( MPA )and 9 cases were Wegeners granulomatosis (WG)and 10 cases were Churg - Strauss syndrome (CSS).71.15 percent (37/52) of these cases were ANCA positive.The most obvious involvement organs were lung and kidney, with 80.77％ and 69.23％ respectively.After the treatments with corticosteroid and cyclophosphamide,46 cases improved,5 cases given up and 1 case died.Conclusion It is very difficult to make diagnosis of primary small vessel vasculitis with involvement of lungs and should be paid more attention to these issues.%目的 提高对原发性小血管炎临床特点和诊断方法的认识.方法 对我院2004年1月～2010年1月确诊的52例原发性小血管炎患者的症状、胸部影像学表现、实验室检查、病理检查结果等进行回顾性分析.结果 52例患者中显微镜下多血管炎33例,韦格纳氏肉芽肿病9例,变应性肉芽肿性血管炎10例;肺脏和肾脏受累最为明显,分别为80.77%和69.23%;抗中性粒细胞胞浆抗体阳性37例(71.15%).应用糖皮质激素和环磷酰胺治疗后好转46例,放弃治疗自动出院5例,死亡1例.结论 原发性小血管炎临床表现复杂多样,诊断困难,误诊率高,应引起临床医师的重视.
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