摘要
IgA肾病是指肾小球系膜区以IgA或IgA沉积为主的一种原发性肾小球疾病.系膜区沉积的IgA主要为多聚体IgA1,多聚体IgA1与抗聚糖抗体形成免疫复合物进而激活补体系统.补体系统在IgA肾病发病机制中主要涉及替代途径和凝集素途径,发挥重要作用.替代途径产物H因子、因子B及凝集素途径产物甘露糖结合凝集素(mannose-binding lectin,MBL)、纤维胶凝蛋白Ficolin、甘露糖结合凝集素相关丝氨酸蛋白酶等参与形成系膜区免疫沉积物,它们在疾病作用尚未阐明.通过抑制补体激活以治疗IgA肾病具有一定潜力,目前已经开展了一系列相关的临床试验.本文就近年来有关补体活化在IgA肾病的进展进行综述.
IgA nephropathy is a primary glomerular disease characterized by IgA or IgA deposition in the glomerular Mesangial region.IgA deposited in the mesangial area is mainly composed of the polymer IgA1,polymeric IgA1 and antiglycan antibody form an immune complex to activate the complement system.Complement system mainly involves alternative pathway and lectin pathway in the pathogenesis of IgA nephropathy and plays an important role.The alternative pathway products of factor H,factor B and lectin pathway products of mannose-binding lectin(mannose-binding lectin,MBL),fibrinogen ficolin and mannose-binding lectin-associated serine protease are involved in the formation of mesangial immune deposits,but their role in the disease has not been elucidated.It has potential to treat IgA nephropathy by inhibiting complement activation,and a series of clinical trials have been carried out.This article reviews the progress of complement activation in IgA nephropathy in recent years.
作者
陈慧萍(综述)
许勇芝
梁东(审校)
CHEN Hui-ping;XU Yong-zhi;LIANG Dong(Institute of Nephrology,the Affiliated Hospital of Guangdong Medical University,Zhanjiang 524001,Guangdong,CHINA)
出处
《海南医学》
CAS
2020年第6期780-783,共4页
Hainan Medical Journal
基金
广东省湛江市财政资金科技专项竞争性分配项目(编号:2016A307)。
关键词
IGA肾病
补体异常
补体抑制剂
临床试验
反义
IgA nephropathy
Complement abnormality
Complement inhibitor
Clinical trial
Antisense
