标题：IgA肾病补体异常的研究进展

      作者：陈慧萍 综述 许勇芝，梁东 审校    广东医科大学附属医院肾脏疾病研究所，广东 湛江 524001

      卷次： 2020年31卷6期

      【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。
      【关键词】 IgA肾病；补体异常；补体抑制剂；临床试验；反义
      【中图分类号】 R692 【文献标识码】 A 【文章编号】 1003—6350（2020）06—0780—04

Research progress of complement abnormalities in IgA nephropathy.
CHEN Hui-ping, XU Yong-zhi, LIANG Dong.Institute of Nephrology, the Affiliated Hospital of Guangdong Medical University, Zhanjiang 524001, Guangdong, CHINA【Abstract】 IgA nephropathy is a primary glomerular disease characterized by IgA or IgA deposition in the glo-merular Mesangial region. IgA deposited in the mesangial area is mainly composed of the polymer IgA1, polymericIgA1 and antiglycan antibody form an immune complex to activate the complement system. Complement system mainlyinvolves alternative pathway and lectin pathway in the pathogenesis of IgA nephropathy and plays an important role. Thealternative pathway products of factor H, factor B and lectin pathway products of mannose-binding lectin (mannose-bind-ing lectin, MBL), fibrinogen ficolin and mannose-binding lectin-associated serine protease are involved in the formationof mesangial immune deposits, but their role in the disease has not been elucidated. It has potential to treat IgA nephropa-thy by inhibiting complement activation, and a series of clinical trials have been carried out. This article reviews theprogress of complement activation in IgA nephropathy in recent years.
      【Key words】 IgA nephropathy; Complement abnormality; Complement inhibitor; Clinical trial; Antisense·综述·doi:10.3969/j.issn.1003-6350.2020.06.030基金项目：广东省湛江市财政资金科技专项竞争性分配项目(编号：2016A307)