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Fibrillary Glomerulonephritis: A Great Mimicker of Rapidly Progressive Glomerulonephritis
1Internal Medicine, Guthrie Robert Packer Hospital, Sayre, USA.
Abstract:
Fibrillary glomerulonephritis (FGN) is a rare but severe kidney disease found to have non-amyloid fibrillary deposits in the mesangium and/or glomerular capillary wall. It was initially thought to be idiopathic, but recent studies show an association with autoimmune disease, malignancy, and hepatitis C infection. We report a case of a non-diabetic patient presenting with long-standing microscopic hematuria, progressive proteinuria, hypertension, and worsening kidney function. The kidney biopsy demonstrated subepithelial fibrillar deposits of size 17 mm randomly oriented with one partial cellular crescent on electron microscopy. Direct immunofluorescence showed no staining for IgG or light chains. It was weakly positive for Congo red staining with a slightly higher serum free kappa/lambda light chain ratio, but serum immunofixation showed no monoclonal protein detection. We empirically treated with rituximab but with no clear benefit or no renal recovery and eventually started on hemodialysis. FGN has an extremely poor prognosis with very few treatment options available. We report this case to emphasize the need for larger, multi-center studies for treatment approaches with collaborating and consolidating data from case reports and case series due to the rarity of the disease.
Insights
Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by abnormal deposits. This case highlights FGN
Area of Science:
- Nephrology
- Pathology
Background:
- Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by non-amyloid fibrillary deposits.
- Previously considered idiopathic, FGN is now associated with autoimmune conditions, malignancies, and hepatitis C.
- Understanding FGN's pathogenesis is crucial for developing effective treatments.
Observation:
- A case of FGN in a non-diabetic patient with microscopic hematuria, proteinuria, hypertension, and declining kidney function.
- Kidney biopsy revealed subepithelial fibrillar deposits and a cellular crescent.
- Immunofluorescence was negative for IgG/light chains; Congo red staining was weakly positive.
Findings:
- Electron microscopy showed characteristic fibrillar deposits.
- Weakly positive Congo red staining and an elevated kappa/lambda ratio suggested potential light chain involvement.
- Rituximab treatment showed no significant benefit, leading to hemodialysis.
Implications:
- FGN has a poor prognosis and limited treatment options.
- This case underscores the need for multi-center studies to explore treatment strategies for FGN.
- Consolidating data from case reports and series is vital for advancing FGN research.
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