胶原纤维性肾小球病中的系膜细胞活化。病例报告及文献复习。

Mesangial cell activation in the collagenofibrotic glomerulonephropathy. Case report and review of the literature.

作者信息

Naruse K, Ito H, Moriki T, Miyazaki E, Hayashi Y, Nakayama H, Kiyoku H, Hiroi M, Kurashige T, Enzan H

机构信息

First Department of Pathology, Kochi Medical School, Japan.

出版信息

Virchows Arch. 1998 Aug;433(2):183-8. doi: 10.1007/s004280050234.

DOI:10.1007/s004280050234

PMID:9737797

Abstract

Collagenofibrotic glomerulonephropathy is a new disease entity of unknown pathogenesis, which is characterized by the deposition of type III collagen within the mesangial matrix. We have investigated a case in which many mesangial cells in the type III collagen-deposited glomeruli were alpha-smooth muscle actin (ASMA) positive and showed an increase of subplasmalemmal filaments, indicating the activation and myofibroblastic transformation. It is suggested that the activated mesangial cells may synthesize the type III collagen deposited in the subendothelial space and mesangial matrix.

摘要

胶原纤维性肾小球病是一种发病机制不明的新疾病实体，其特征是Ⅲ型胶原在系膜基质内沉积。我们研究了一例病例，在Ⅲ型胶原沉积的肾小球中有许多系膜细胞α平滑肌肌动蛋白（ASMA）呈阳性，并且显示出质膜下细丝增加，表明细胞被激活并发生肌成纤维细胞转化。提示被激活的系膜细胞可能合成沉积在内皮下间隙和系膜基质中的Ⅲ型胶原。