【摘要】 目的探讨原发性系统性小血管炎的临床病理特点和诊断,以提高对本病认识。方法对2001年-2006年本院肾内科确诊的10例原发性系统性小血管炎临床资料进行分析,探讨原发性系统性小血管炎患者的临床病理特点。结果10例患者男3例,女7例年龄24～77岁,平均55.4岁。3例ANCA阴性,7例IIF-ANCA阳性。10例均有肾脏损害,表现为血尿、蛋白尿、肾功能损害和病理改变。5例有肺受累,6例有听力损害。均有贫血,血沉增快和CRP明显升高。所有患者确诊时间均较长。结论系统性小血管炎并不少见;临床表现呈多系统损害,肺、肾、血液系统及听力损害最常见;ANCA可作为诊断的特异性指标。更多还原

【Abstract】 Objective To investigate the clinicopathological characteristics and diagnosis of primary systemic small vasculitis. Methods The retrospective analysis of clinical materials was performed on 10 patients with primary systemic small vasculitis in our hospital from 2001 to 2007. Results Among the 10 patients, there were 3 male and 7 female.Their ages range from 24 to 77.The main clinicopathological characteristics included ANCA(antineutrophil cytoplasmic antibody) negative (3 cases),ANCA positive (7 cases),renal damage(10 cases) including haematuria,proteinuria,renal fuction damage and pathological damage,lung damage (5 cases), acouesthesia damage(6cases), anaemia(10 cases), rapid ESR and elevated CRP (10 cases), Conclusion Primary systemic small vasculitis is not rare in China. Its characteristics appears multi-system involvement,specially with lung, kidney,blood system and acouesthesia; ANCA can be used as a specific index for diagnosis.更多还原