Fibrillary glomerulonephritis: presenting as crescentic glomerulonephritis causing rapidly progressive renal failure.

NDT Plus Pub Date : 2011-12-01 DOI:10.1093/ndtplus/sfr146
Shalini Nilajgi, John Paul Killen, Richard Baer, Patricia Renaut, Murty Mantha
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引用次数: 7

Abstract

We report an unusual case of fibrillary glomerulonephritis (FGN) presenting as rapidly progressive renal failure and extensive crescent formation along with linear staining of capillary walls of the glomeruli on immunofluorescence, mimicking anti-glomerular basement membrane (anti-GBM) antibody-mediated disease. Laboratory results for circulating anti-GBM antibodies were negative. The subsequent electron microscopic findings were that of presence of electron-dense deposits in the glomerular mesangium and capillary walls, comprising of non-branching fibrils with an average diameter of 16 nm consistent with a diagnosis of FGN. This case illustrates the crucial role of electron microscopy in differential diagnosis of crescentic glomerulonephritis.

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原纤维性肾小球肾炎:表现为新月形肾小球肾炎,引起迅速进行性肾衰竭。
我们报告一例罕见的原纤维性肾小球肾炎(FGN),表现为快速进展性肾衰竭和广泛的新月形成,免疫荧光显示肾小球毛细血管壁呈线性染色,模拟抗肾小球基底膜(抗gbm)抗体介导的疾病。循环抗gbm抗体实验室结果为阴性。随后的电镜检查结果显示肾小球系膜和毛细血管壁存在电子致密沉积物,由平均直径为16 nm的非分枝原纤维组成,与FGN的诊断一致。本病例说明了电子显微镜在鉴别诊断月牙状肾小球肾炎中的重要作用。
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