儿童抗中性粒细胞胞浆抗体相关性血管炎3例报告并文献复习

Anti-neutrophil cytoplasmic antibody-related vasculitis in children : a report of 3 cases and literature review

目的探讨儿童抗中性粒细胞胞浆抗体(ANCA)相关性血管炎的临床特征以及诊断和治疗。方法回顾分析2013年至2017年确诊的3例ANCA相关性血管炎患儿的临床资料。结果 1例为男性(例1,2岁),2例为女性(例2,5岁;例3,17岁)。3例患儿的首发表现不同,例2、例3有不同程度肾脏损伤,例1、例2有不同程度肺损伤,例1皮肤及肝脏受累。例2的MPO、P-ANCA阳性,经治疗1年后复查阴性,后因肺部感染再次P-ANCA阳性;例1、例3的ANCA相关抗体均阴性。例2患儿经过激素联合环磷酰胺冲击治疗后明显好转,病程中因感染复发及经济问题自动出院失访;例1、例3患儿经过激素、支持等治疗后,病情好转出院。讨论儿童ANCA相关性血管炎的首发临床表现不一,当疾病累及肾脏、肺部等多器官时,需警惕ANCA相关性血管炎,早诊断、早治疗可以改善其预后。

Objective To explore the clinical features,diagnosis and treatment of Anti-neutrophil cytoplasmic antibody associated vasculitis(ANCA)in children,and raise awareness of the disease.Methods The clinical data of 3 children with ANCA associated vasculitis were retrospectively analyzed from 2013 to 2017 in the hospital.Results Of the 3 cases,2 were girls and 1 boys.One case was positive for MPO,one case was positive for P-ANCA,one case was negative for P-ANCA after one year of treatment,and the other two cases were negative for ANCA related antibodies because of pulmonary infection.The initial performance of 3 cases was different.2 cases had different degrees of renal injury,2 cases had different degrees of lung injury,1 case had skin involvement,and 1 case had liver involvement.In case 2,after the therapy of glucocorticoid combined with cyclophosphamide,the patient had improved significantly.However,the patient was automatically discharged from hospital due to recurrence of infection and economic problems during the course of the disease.Cases 1 and 3 were treated with hormones,support,etc.,and their condition improved and discharged.Conclusions The first clinical manifestation of ANCArelated vasculitis in children vary widely,and when multiple organs such as kidney and lungs are damaged,we should be alert to ANCA-related vasculitis,and early diagnosis and early treatment can improve their prognosis.