目的 探讨抗中性粒细胞胞浆抗体(ANCA)相关小血管炎的临床表现.方法 回顾性分析3例ANCA相关小血管炎患儿的临床资料.结果 3例患儿均为女性.2例环核型ANCA (P-ANCA)、抗髓过氧化物酶(MPO)抗体均为阳性；1例P-ANCA阳性,但未作MPO检查.3例患儿均出现肾损害,其中例1患儿出现严重肺出血.例1患儿接受大剂量甲基泼尼松龙及环磷酰胺治疗,但因发生慢性肾衰竭而只能维持肾脏替代治疗；例2经皮质激素联合吗替麦考酚酯治疗,病情得到控制；例3停用丙基硫氧嘧啶并予小剂量激素口服,病情得到控制.结论 儿童ANCA相关性血管炎以女性多见,以P-ANCA阳性为主.早期诊断、合理使用皮质激素及联合免疫抑制剂是改善其预后的重要措施.%Objective To explore the clinical features of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Methods The clinical records of 3 children with ANCA-associated vasculitis who were hospitalized after 2007 were retrospectively reviewed. Results The 3 children were all girls. Two children were both perinuolear ANCA (P-ANCA) and myeloperoxidase ANCA (MPO-ANCA) positive by serologieal test. Another child was P-ANCA positive, but MPO-ANCA was not checked. All 3 children suffered with kidney damage. Furthermore, case 1 showed severe pulmonary hemorrhage. After the treatment, although the fatal pulmonary hemorrhage was resolved, case 1 displayed chronic kidney disease which necessitated renal replacement therapy. Case 2 entered into remission by the treatment of steroids combined with Mycophenolale Mofetil. Case 3 also entered into remission by withdraw of propylthi-ouracil and resorting to low-dose steroids. Conclusions Girls account for a large part of ANCA-associated vasculitis in children, and the serologieal results was most likely to be P-ANCA positive. Early diagnosis, rational use of corticos-teroids and immunosuppressive agents are important measures to improve the prognosis of ANCA-associated vasculitis.
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