Pathogenesis of IgA nephropathy.
Pathogenesis of IgA nephropathy.
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DOI：
10.1053/s0270-9295(03)00134-7
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发表时间：
2003-11
影响因子：
3.3
通讯作者：
J. Wada;H. Sugiyama;H. Makino
中科院分区：
文献类型：
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作者：
J. Wada;H. Sugiyama;H. Makino
免疫球蛋白A(IgA)肾病是一种免疫复合体介导的肾小球肾炎，其特征是免疫球蛋白A沉积在系膜和系膜旁区域。IgA肾病患者具有不同的临床症状(如微量血尿并保留肾功能或肾功能进行性恶化导致终末期肾病)。参与IgA肾病发病机制的因素有：(1)。环境因素，(2)。遗传因素，(3)。(4)IgA1分子异常。各种炎症介质。人类白细胞抗原(HL A)、肾素-血管紧张素-醛固酮系统和选择素基因簇的基因多态性研究表明，IgA肾病患者存在一定程度的遗传易感性。此外，家族性IgA肾病的全基因组筛查显示，IgA肾病与6q22-23染色体连锁。除了在其发病机制中受遗传因素的影响外，在IgA肾病患者肾脏中还观察到血清中IgA和IgA1的异常半乳糖化，这种异常可能导致多种细胞因子、白介素6、血小板衍生生长因子、肿瘤坏死因子-α和转化生长因子-β1在肾细胞中的表达，从而导致进一步的肾小球损伤。尽管文献中提供了大量的信息，但仍需要进一步的研究来描述与原发性IgA肾病有关的确切发病机制，并促进新的治疗干预措施的发展。
Immunoglobulin A (IgA) nephropathy is an immune-complex-mediated glomerulonephritis characterized by the presence of immunoglobulin A deposits in mesangial and paramesangial regions. The patients with IgA nephropathy present with varying clinical symptoms (eg, microhematuria with preserved renal function or progressive deterioration of renal functions resulting in end-stage renal disease). The factors involved in the pathogenetic mechanisms of IgA nephropathy include (1). environmental factors, (2). genetic factors, (3). abnormality of the IgA1 molecule, and (4). various inflammatory mediators. The gene polymorphism studies for human leukocyte antigen (HLA), renin-angiotensin-aldosterone system, and selectin gene clusters, suggest a certain degree of genetic predisposition in patients for IgA nephropathy. Also, the genome-wide screening in familial IgA nephropathy showed linkage of IgA nephropathy to the 6q22-23 chromosome. Besides genetic influence in its pathogenesis, aberrant galactosylation in serum IgA and IgA1 eluted from kidneys with IgA nephropathy has been observed, and conceivably such abnormalities induce the expression of various cytokines, interleukin (IL)-6, platelet-derived growth factor (PDGF), tumor necrosis factor (TNF)-alpha, and transforming growth factor (TGF)-beta1 in the renal cells, which contributes to further glomerular injury. Despite an enormous amount of information available in the literature, further studies are needed to delineate the precise pathogenetic mechanisms involved in primary IgA nephropathy and also to facilitate the development of newer therapeutic interventions.