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[IgA肾病的新免疫致病机制]

[New immunopathogenic aspects of IgA nephropathy].

作者信息

Hernando L, González-Cabrero J, García-Hoyo R, Hernando P, Egido J

机构信息

Fondation Jiménez Díaz, Université Autonoma, Madrid, Espagne.

出版信息

Bull Acad Natl Med. 1990 May;174(5):549-59.

PMID:2249143
Abstract

IgA nephropathy (Berger's disease) has become recognized worldwide as one of the most common of the primary glomerulonephritis. The mesangial granular deposits suggested an immune complex disease. The available data evidence that the IgA circulating immune complexes in these patients are heterogeneous. Recent analysis, performed after dissociating the complexes, found both IgA1 and IgG. In fact, high serum levels of IgA rheumatoid factor and shared antibody idiotypes were found in a large proportion of those patients. A close relationship was noted between the presence of cross-reactive idiotypes on mesangial immunoglobulins and the existence of increase levels of serum idiotypes and many patients have increased rates of IgA synthesis either spontaneously or after stimulation of the peripheral blood mononuclear cells by various mitogens. A lot of abnormalities on B and T lymphocytes, related with the IgA immune regulation, have been described. Most of deposited IgA in the mesangium is polymeric and belongs to the IgA1 class. Patients with IgA nephropathy have very often antibodies against exogenous and endogenous antigens. Among the most frequently found are the antibodies against dietary, viral and bacterial antigens as well as against the Fc and Fab portions of immunoglobulins, nuclear and glomerular antigens. The mechanisms of mesangial damage in IgA nephropathy are not well known. Mesangial cells are capable of producing and releasing various lipidic and proteic mediators. The stimulation of mesangial cells, cultured in vitro by IgA or IgG immune complexes induced the release of PAF, PGE2 and superoxide anion. A better knowledge of the mechanism implicated in the abnormality of IgA immune regulation, as well as of the glomerular inflammation response could afford a new therapeutic approach to this nephropathy.

摘要

IgA肾病(伯杰氏病)已被公认为全球最常见的原发性肾小球肾炎之一。系膜颗粒沉积物提示为免疫复合物疾病。现有数据表明,这些患者循环中的IgA免疫复合物是异质性的。最近在解离复合物后进行的分析发现了IgA1和IgG。事实上,在这些患者中很大一部分发现了高血清水平的IgA类风湿因子和共享抗体独特型。系膜免疫球蛋白上交叉反应独特型的存在与血清独特型水平升高之间存在密切关系,许多患者自发或在各种有丝分裂原刺激外周血单个核细胞后IgA合成率增加。已经描述了许多与IgA免疫调节相关的B和T淋巴细胞异常。系膜中沉积的大多数IgA是多聚体,属于IgA1类。IgA肾病患者经常有针对外源性和内源性抗原的抗体。最常见的是针对饮食、病毒和细菌抗原以及免疫球蛋白的Fc和Fab部分、核抗原和肾小球抗原的抗体。IgA肾病中系膜损伤的机制尚不清楚。系膜细胞能够产生和释放各种脂质和蛋白质介质。用IgA或IgG免疫复合物在体外培养刺激系膜细胞可诱导PAF、PGE2和超氧阴离子的释放。更好地了解与IgA免疫调节异常以及肾小球炎症反应相关的机制,可能为这种肾病提供一种新的治疗方法。

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