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Fibrillary glomerulonephritis: presenting as crescentic glomerulonephritis causing rapidly progressive renal failure
Shalini Nilajgi1, John Paul Killen1, Richard Baer1
1Department of Renal Medicine, Cairns Base Hospital, Cairns, Australia.
NDT Plus
|May 19, 2015
Summary
Fibrillary glomerulonephritis mimicked anti-GBM disease with rapid kidney failure. Electron microscopy confirmed FGN by revealing characteristic fibrils, crucial for diagnosing crescentic glomerulonephritis.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Fibrillary glomerulonephritis (FGN) is a rare kidney disease.
- Rapidly progressive renal failure with crescent formation can mimic other glomerular diseases.
Observation:
- A case presented with rapid renal failure and glomerular crescents, suggesting anti-glomerular basement membrane (anti-GBM) disease.
- Immunofluorescence showed linear capillary wall staining, further supporting anti-GBM disease.
- Circulating anti-GBM antibodies were negative.
Findings:
- Electron microscopy revealed electron-dense deposits in glomeruli.
- These deposits contained non-branching fibrils (16 nm diameter), characteristic of FGN.
- This confirmed FGN despite initial presentation mimicking anti-GBM disease.
Implications:
- Electron microscopy is vital for differentiating FGN from anti-GBM disease in crescentic glomerulonephritis.
- Accurate diagnosis impacts patient management and prognosis.
- Highlights the importance of advanced imaging in complex renal pathology.
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