膜性肾病:问题的现状

O. Sharapov, S. Abdullaev
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摘要

膜性肾病(MN)是肾小球自身免疫性疾病,是肾病综合征的主要原因之一。该病表现出异质性结局,约30%的病例进展为终末期肾脏疾病。由于2009年发现了磷脂酶A2受体(PLA2R)自身抗体,2014年发现足细胞表面含有血栓反应蛋白结构域7A (THSD7A), MN发病机制的研究稳步推进。大约50 - 80%和3 - 5%的原发性MN病例分别与抗pla2r或抗thsd7a抗体相关。这些自身抗体的存在可用于MN诊断;抗体水平与疾病严重程度相关,在监测疾病进展和治疗反应方面具有重要的生物标志物价值。
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Membranous nephropathy: the current state of the problem
Membranous nephropathy (MN) is an autoimmune disease of the kidney glomeruli and one of the leading causes of nephrotic syndrome. The disease exhibits heterogenous outcomes with approximately 30 % of cases progressing to end-stage renal disease. The study of MN pathogenesis has steadily advanced owing to the identification of autoantibodies to the phospholipase A2 receptor (PLA2R) in 2009 and thrombospondin domain-containing 7A (THSD7A) on the podocyte surface in 2014. Approximately 50–80 and 3–5 % of primary MN cases are associated with either anti-PLA2R or anti-THSD7A antibodies, respectively. The presence of these autoantibodies is used for MN diagnosis; antibody levels correlate with disease severity and possess significant biomarker values in monitoring disease progression and treatment response.
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