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Am J Kidney Dis. 2012 Jul;60(1):157-9. doi: 10.1053/j.ajkd.2011.12.024. Epub 2012 Mar 6.

Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis.

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  • 1Johns Hopkins Medical Institutions, Baltimore, MD, USA.


Fibrillary glomerulonephritis (GN) is an uncommon cause of rapidly progressive kidney failure. We report a case of rapidly progressive kidney failure with kidney biopsy showing crescentic GN on light microscopy and immunofluorescence showing linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G and C3, consistent with anti-GBM disease. However, electron microscopy showed fibrillary deposits in the GBM, suggesting a diagnosis of fibrillary GN. As exemplified by this case, it is important to consider fibrillary GN in the differential diagnosis of crescentic GN with linear immunoglobulin G deposits within the GBM. Electron microscopy is crucial to make this diagnosis.

Copyright © 2012 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

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