IgA肾病的发病机制及治疗进展

摘要

Immunoglobulin A nephropathy(IgAN) is the most common primary glomerular disease.IgAN is not benign.20%-30% of them develops into end-stage renal disease in 10-20 years.IgAN patients having high level of intrinsic galactose deficient IgA1, specific IgG binding with Gd-IgA1 and depositing in the mesangial area,triggering the inflammatory immune response against the kidney are three steps of the patho-genesis of IgAN.IgAN has many pathological types and clinical manifestations,however there is no specific treatment for the time being,and the clinical goal is to reduce urinary protein,control blood pressure,sup-press the immune response,and so on,so as to reduce renal damage and delay the progression.%IgA肾病（ IgAN）是最常见的原发性肾小球疾病。 IgAN并非良性疾病，20％～30％的患者10～20年后进展至终末期肾脏疾病。 IgAN患者体内高水平的半乳糖缺乏的IgA1（Gd－IgA1）水平、特异性的IgG与Gd－IgA1结合并沉积于系膜区、触发免疫炎症反应攻击肾脏是 IgAN发病的3个步骤。IgAN病理类型及临床表现多样。目前无特异性的治疗药物，临床治疗目标是通过降低尿蛋白、控制血压、抑制免疫反应等措施减轻肾组织进一步损伤，延缓疾病进展。