摘要
免疫球蛋白A(IgA)沉积于肾小球是IgA肾病的特点,是非常普遍的现象。IgA亚类(IgA1)的异常O-联半乳糖基化在这个过程中起到主要作用,针对IgA1铰链区聚多糖相关表位的自身免疫是必要的发病条件。已证明既存在循环免疫复合物也存在原位免疫复合物。先天免疫素质及中介系统,如补体系统也在临床发病及病情演变中起重要作用。对于IgA肾病详细机制的理解有助于改善诊断方法,也有助于对这一常见的原发性全球分布疾病做出更加精确的个性化预后预测及个性化治疗方法的改进。
Deposition of IgA in the glomeruli, the hallmark of IgA nephropathy,is a quite common phe-nomenon. Aberrant O-linked galactosylation of IgA subclass （ IgA1） appears to play a central role in the process and auto-immunity to a conformational epitope related to glycans at the hinge region of IgA1 is apparently required. Both circulating immune complex and in-situ immune complex mechanism have been proven to exist. Mediator systems, such as complement activation and engagement of innate immune system, also play prominent roles in determining the clinical onset and severity of disease. Progress in understanding the details of the pathogenesis of IgA nephropathy will lead to diagnosis improvement, more accurate individualized prog-nosis and personalized treatment regimens for this globally distributed and very common primary disease.
出处
《医学综述》
2012年第15期2394-2397,共4页
Medical Recapitulate
