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Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis.

H H Shah1, J Thakkar1, J M Pullman2

  • 1Department of Medicine, Division of Kidney Diseases and Hypertension, North Shore University Hospital and Long Island Jewish Medical Center, Hofstra Northwell School of Medicine, Great Neck, USA.

Indian Journal of Nephrology
|March 31, 2017
PubMed
Summary

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Fibrillary glomerulonephritis (FGN) rarely presents as crescentic glomerulonephritis. This rare form, crescentic FGN, often leads to severe kidney failure, with limited treatment options and poor outcomes, even with immunosuppression.

Area of Science:

  • Nephrology
  • Pathology

Background:

  • Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease.
  • It typically presents with hypertension, proteinuria, hematuria, and renal insufficiency.
  • Histologically, FGN shows mesangioproliferative, membranoproliferative, or membranous patterns.

Observation:

  • Crescent formation is an uncommon finding in FGN.
  • The crescentic glomerulonephritis pattern in FGN is rarely described.
  • This case report details an adult patient with massive proteinuria and severe renal failure.

Findings:

  • Kidney biopsy confirmed crescentic FGN (C-FGN).
  • The patient required long-term dialysis despite immunosuppressive therapy.
  • Literature review indicates C-FGN often presents as rapidly progressive or advanced renal failure.
Keywords:
Crescentic glomerulonephritisfibrillary glomerulonephritisglomerulonephritisrapidly progressive glomerulonephritis

Related Experiment Videos

Implications:

  • Optimal therapy and outcomes for crescentic FGN are currently unknown.
  • This rare presentation of FGN poses significant management challenges.
  • Further research is needed to understand and treat C-FGN effectively.