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

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.