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Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis
Purva Sharma1, Michael Kuperman, Lorraine Racusen
1Johns Hopkins Medical Institutions, Baltimore, MD, USA. psharm16@jhmi.edu
Summary
Fibrillary glomerulonephritis (GN) is a rare cause of kidney failure. This case highlights the importance of electron microscopy in diagnosing fibrillary GN, especially when initial tests suggest anti-GBM disease.
Area of Science:
- Nephrology
- Pathology
Background:
- Fibrillary glomerulonephritis (GN) is an uncommon cause of rapidly progressive kidney failure.
- Distinguishing fibrillary GN from other crescentic GN variants can be challenging.
Observation:
- A case of rapidly progressive kidney failure presented with crescentic GN on light microscopy.
- Immunofluorescence showed linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G (IgG) and C3, mimicking anti-GBM disease.
Findings:
- Electron microscopy revealed fibrillary deposits within the GBM, confirming a diagnosis of fibrillary GN.
- This finding underscores the limitations of light microscopy and immunofluorescence alone in specific cases.
Implications:
- Fibrillary GN should be considered in the differential diagnosis of crescentic GN with linear IgG deposits.
- Electron microscopy is essential for accurate diagnosis and appropriate patient management.
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