儿童抗中性粒细胞胞浆抗体相关小血管炎3例分析  

Clinical features of three cases of children with anti-neutrophil cytoplasmic antibody associated vasculitis

作　　者：姚盛华[1] 毛建华[1] 傅海东[1] 方澄清[1] 沈辉君[1] 刘爱民[1] 

机构地区：[1]浙江大学医学院附属儿童医院肾内科,浙江杭州310006

出　　处：《临床儿科杂志》2012年第4期312-315,共4页Journal of Clinical Pediatrics

基　　金：国家自然科学基金资助项目(No.30971365;81070561;81170664);卫生部科学研究基金-浙江省医药卫生重大科技计划(No.WKJ2010-2-014)

摘　　要：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 perinuclear ANCA（P-ANCA）and myeloperoxidase ANCA（MPO-ANCA）positive by serological 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 Mycophenolate 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 serological results was most likely to be P-ANCA positive.Early diagnosis,rational use of corticosteroids and immunosuppressive agents are important measures to improve the prognosis of ANCA-associated vasculitis.

关 键 词：抗中性粒细胞胞浆抗体 血管炎 激素 免疫抑制剂 儿童 

分 类 号：R725.9[医药卫生—儿科]