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伴有IgA沉积为主的纤维性肾小球肾炎合并未分化结缔组织病:一例报告

Fibrillary glomerulonephritis with prevalent IgA deposition associated with undifferentiated connective tissue disease: A case report.

作者信息

Nebuloni Manuela, Genderini Augusto, Tosoni Antonella, Caruso Sabrina, di Belgiojoso Giovanni Barbiano

机构信息

Nephrology Unit, 'L.Sacco' Hospital , Milan , Italy.

Pathology Unit, L. Sacco Hospital.

出版信息

NDT Plus. 2010 Feb;3(1):57-9. doi: 10.1093/ndtplus/sfp125. Epub 2009 Sep 19.

Abstract

We described a 41-year-old female patient, who presented with proteinuria occurring 5 years after the onset of an undifferentiated connective tissue disease (UCTD). At renal biopsy, a pattern of focal necrotizing glomerulonephritis with mesangial and parietal deposition of the IgA, C3 and K chains was observed. Electron microscopy showed organized fibrillary deposits in mesangial, subendothelial, intramembranous and subepithelial sites. Fibrils were randomly arranged, had no hollow core and had a diameter ranging between 10 and 23 nm. This case showed a rare combination of fibrillary glomerulonephritis and prevalent IgA deposition, in the clinical context of UCTD.

摘要

我们描述了一名41岁女性患者,她在未分化结缔组织病(UCTD)发病5年后出现蛋白尿。肾活检时,观察到局灶性坏死性肾小球肾炎的模式,伴有IgA、C3和κ链在系膜和壁层的沉积。电子显微镜显示在系膜、内皮下、膜内和上皮下部位有有组织的纤维状沉积物。纤维随机排列,无中空核心,直径在10至23纳米之间。在UCTD的临床背景下,该病例显示了纤维性肾小球肾炎和IgA普遍沉积的罕见组合。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9b7c/4421553/ca5244d179a4/sfp125fig1.jpg

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