Fibrillary Glomerulonephritis: A Great Mimicker of Rapidly Progressive Glomerulonephritis

Manisha Raikar1, Asad Shafiq2

  • 1Internal Medicine, Guthrie Robert Packer Hospital, Sayre, USA.

Cureus
|July 22, 2022
PubMed

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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