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膜性肾小球病:不断演变的故事。

Membranous glomerulopathy: the evolving story.

机构信息

INSERM UMR_S 702, Paris, France.

出版信息

Curr Opin Nephrol Hypertens. 2010 May;19(3):254-9. doi: 10.1097/MNH.0b013e328336eafd.

Abstract

PURPOSE OF REVIEW

Membranous nephropathy is one of the most common glomerulopathies. Current treatments are entirely empirical, and pathophysiology-driven therapies are dramatically lacking. This review focuses on new pathophysiologic aspects of the disease, with special emphasis on the antigenic targets of pathogenic antibodies.

RECENT FINDINGS

In the past few years, two major antigens have been identified in human membranous nephropathy. The first is neutral endopeptidase (NEP), the alloantigen involved in neonatal cases of membranous nephropathy that occur in newborns from NEP-deficient mothers. The second is the M-type phospholipase A2 receptor (PLA2R), the first autoantigen identified in idiopathic membranous nephropathy in the adult.

SUMMARY

A common denominator shared by most cases of membranous nephropathy is the expression by podocytes of antigenic targets for in-situ formation of glomerular immune deposits. The recent discovery of neutral endopeptidase and PLA2R provides new tools for the monitoring of disease activity and should be of value in designing new antigen-driven therapeutic strategies.

摘要

目的综述

膜性肾病是最常见的肾小球疾病之一。目前的治疗方法完全是经验性的,缺乏基于病理生理学的治疗方法。本综述重点介绍了该疾病的新病理生理方面,特别强调了致病性抗体的抗原靶点。

最近的发现

在过去的几年中,已经在人类膜性肾病中确定了两种主要的抗原。第一种是中性内肽酶(NEP),它是与母体内缺乏 NEP 的新生儿发生的新生儿膜性肾病有关的同种异体抗原。第二种是 M 型磷脂酶 A2 受体(PLA2R),它是成人特发性膜性肾病中鉴定的第一个自身抗原。

总结

大多数膜性肾病的共同点是足细胞表达原位形成肾小球免疫沉积物的抗原靶点。最近发现的中性内肽酶和 PLA2R 为疾病活动的监测提供了新的工具,并且应该对设计新的基于抗原的治疗策略具有价值。

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