抗中性粒细胞胞浆抗体相关性小血管炎30例临床诊治分析

目的：探讨抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎的临床特点、诊断和治疗。方法：回顾性分析2002年6月~2009年6月检测并明确诊断的30例ANCA相关性小血管炎患者的临床病理资料。结果：30例患者中胞浆型ANCA（c-ANCA）阳性4例,3例识别蛋白酶3（PR3）,1例识别髓过氧化物酶（MPO）;核周型ANCA（p-ANCA）阳性26例,均识别MPO。临床表现呈多器官受累,以肾、肺受累为主。多数患者有贫血、血沉增快和C反应蛋白增高。糖皮质激素联合免疫抑制剂治疗,诱导缓解的缓解率为83.3%。结论：ANCA相关性小血管炎临床表现为多器官受累,缺乏特异性,其诊断要结合临床表现、ANCA检测和病理活检综合考虑,糖皮质激素联合免疫抑制剂治疗有较好疗效,吗替麦考酚酯和硫唑嘌呤等免疫抑制剂较环磷酰胺毒副作用更小。

Analysis of Clinical Diagnostic and Therapeutic Characteristics of 30 Patients with Anti-neutrophil Cytoplasmic Autoantibodies Related Vasculitis

Objective:To evaluate the clinical characteristics of diagnosis and treatment in anti-neutrophil cytoplasmic autoantibodies(ANCA) associated systemic vasculitis.Methods: Thirty patients with ANCA accociated systemic vasculitis diagnosed from 2002 to 2009 were retrospectively analyzed with pathological information.Results:Of the 30 patients,4 were cytoplasmic ANCA(c-ANCA) positive;3 were recognized proteinase 3(PR3);1 was recognized myeloperoxidase(MPO);26 were perinuclear ANCA(p-ANCA) positive;and all of th...