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机构地区:[1]解放军总医院肾脏病科解放军肾脏病研究所肾脏疾病国家重点实验室国家慢性肾病临床医学研究中心,北京100853
出 处:《解放军医学院学报》2017年第6期550-555,共6页Academic Journal of Chinese PLA Medical School
基 金:国家重点基础研究发展计划(973项目)(2014CBA02005)~~
摘 要:IgA肾病在全球范围内是最常见的原发性肾小球疾病。其最显著的组织病理学特点是以IgA为主的免疫复合物沉积于肾小球系膜区,诱发系膜细胞增殖、分泌各种炎性因子及趋化因子介导肾小球炎症损伤。目前IgA肾病的确诊仍依赖于肾穿刺活检。本文综述了IgA肾病发病机制的进展,包括异常糖基化IgA1,针对异常糖基化IgA1的自身抗体的产生,IgA循环免疫复合物的形成和在肾小球系膜区沉积,对由IgA免疫复合物诱导炎症损伤过程中的关键分子事件等在近年来取得的研究进展进行介绍,包括了对亚、欧、美洲等不同种族人群IgA肾病患者大规模的全基因组关联研究进展。此外,也对近年来IgA肾病诊断及预后相关的生物标记物的研究进行了介绍。IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide. The hallmark of the disease is that IgA immune complexes predominantly deposit in the glomerular mesangium, and the IgA containing deposition induces activation and proliferation of mesangial cells which release inflammatory cytokines and chemokines to mediate inflammation in glomerulus. Diagnosis of IgAN still depends on renal biopsy. In this review, we introduce advances in the pathogenesis of IgAN including the key molecular events in generation of aberrantly glycosylated IgA1 and autoantibodies to them, the formation of circulating IgA immune complexes, deposition of immune complexes in the glomerular mesangium, and inflammation in glomerulus induced by IgA immune complexes, as well as the large scale genome-wide association studies in patients with IgAN from Asian, European, and American populations. At last, recent studies on the emerging biomarkers with diagnostic and prognostic potential for IgAN will be introduced.
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