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膜性肾病的免疫发病机制:最新进展

Immunopathogenesis of membranous nephropathy: an update.

作者信息

Debiec Hanna, Ronco Pierre

机构信息

Sorbonne Universités, UPMC Univ Paris 06, UMR_S 1155, 75005, Paris, France,

出版信息

Semin Immunopathol. 2014 Jul;36(4):381-97. doi: 10.1007/s00281-014-0423-y. Epub 2014 Apr 9.

Abstract

Membranous nephropathy (MN) is a non-inflammatory organ-specific autoimmune disease which affects the kidney glomerulus, resulting in the formation of immune deposits on the outer aspect of the glomerular basement membrane, complement-mediated proteinuria, and severe renal failure in 30% of patients. In the last 10 years, substantial advances have been made in the understanding of the molecular bases of MN, with the identification of several antigens and predisposing genes in children and adults. These ground-breaking findings already have a major impact on diagnosis and monitoring and to some extent on therapies. However, there is evidence that the disease is more complex and involves a variety of antigen-antibody systems and genes involved in immune response, progression, recovery, and protective mechanisms. We herein review these recent findings which open new perspectives of research. Understanding the complex pathogenesis of MN will offer many opportunities for future therapeutic interventions and will hopefully have a major impact on patient care. New insights into the molecular mechanisms of MN may also enlighten the pathogenesis of organ-specific autoimmune diseases.

摘要

膜性肾病(MN)是一种非炎性器官特异性自身免疫性疾病,它会影响肾小球,导致在肾小球基底膜外侧形成免疫沉积物、补体介导的蛋白尿,并且30%的患者会出现严重肾衰竭。在过去十年中,在对MN分子基础的理解方面取得了重大进展,已在儿童和成人中鉴定出几种抗原和易感基因。这些开创性的发现已经对诊断和监测产生了重大影响,并在一定程度上影响了治疗。然而,有证据表明该疾病更为复杂,涉及多种抗原-抗体系统以及参与免疫反应、疾病进展、恢复和保护机制的基因。我们在此回顾这些开启新研究视角的最新发现。了解MN复杂的发病机制将为未来的治疗干预提供许多机会,并有望对患者护理产生重大影响。对MN分子机制的新见解也可能为器官特异性自身免疫性疾病的发病机制提供启示。

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