儿童特发性膜性肾病诊疗进展

Progress in diagnosis and treatment of idiopathic membranous nephropathy in children

膜性肾病(MN)是一种免疫复合物介导的肾小球疾病,大多表现为肾病综合征。儿童MN多为继发性MN,最常继发于乙肝病毒感染或系统性红斑狼疮,近年来儿童特发性MN的发病率有增加趋势。M型磷脂酶A2受体(PLA2R)抗体和1型血小板反应蛋白7A域(THSD7A)抗体最初在成人中发现,可作为成人特发性MN诊断、判断疾病活动、评估预后转归、评价治疗效果的生物标志物,目前也成为诊断和监测儿童MN的重要指标。对非肾病水平蛋白尿且病情进展风险较低者,推荐使用保守支持治疗;对持续性大量蛋白尿,伴严重并发症或肾功能进行性恶化者应给予免疫抑制剂治疗。文章综述儿童特发性MN的诊断及其最新的治疗进展。

Membranous nephropathy(MN)is an immune complex mediated glomerular disease and mostly manifests as nephrotic syndrome.In the pediatric population,the MN in children is more often secondary to hepatitis B virus infection or systemic lupus erythematosus.In recent years,the incidence of pediatric idiopathic MN is increasing.M-type phospholipase A2 receptor(PLA2R)and thrombospondin type-1 domain-containing 7A(THSD7A)antibody were initially found in adults.They can be used as biomarkers for the diagnosis of adult idiopathic MN,judgment of disease activity,evaluation of prognosis and assessment of therapeutic effect.Now they are also important indicators for the diagnosis and monitoring of pediatric MN.Conservative support is recommended for patients with non-nephrotic level of proteinuria and low risk of disease progression;immunosuppressive therapy should be given to patients with persistent massive proteinuria,severe complications or deterioration of renal function.This paper reviews the diagnosis and treatment of idiopathic membranous nephropathy in children.