Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis

H. H. Shah, J. Thakkar, J. Pullman, A. T. Mathew

Research output: Contribution to journalReview article

Abstract

Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described. Optimal therapy and outcomes in FGN presenting with crescentic GN is not currently known. We report an adult patient who presented with massive proteinuria and severe renal failure. The kidney biopsy revealed crescentic FGN (C-FGN). The patient remained dialysis dependent despite immunosuppressive therapy. We also briefly review FGN, and the few reported cases of C-FGN that presented as rapidly progressive or advanced renal failure in the literature.

Original languageEnglish (US)
Pages (from-to)157-160
Number of pages4
JournalIndian Journal of Nephrology
Volume27
Issue number2
DOIs
StatePublished - Mar 1 2017

Fingerprint

Glomerulonephritis
Renal Insufficiency
Proteinuria
Kidney
Biopsy
Membranous Glomerulonephritis
Wounds and Injuries
Hematuria
Immunosuppressive Agents
Dialysis
Hypertension
Therapeutics

Keywords

  • Crescentic glomerulonephritis
  • fibrillary glomerulonephritis
  • glomerulonephritis
  • rapidly progressive glomerulonephritis

ASJC Scopus subject areas

  • Nephrology

Cite this

Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis. / Shah, H. H.; Thakkar, J.; Pullman, J.; Mathew, A. T.

In: Indian Journal of Nephrology, Vol. 27, No. 2, 01.03.2017, p. 157-160.

Research output: Contribution to journalReview article

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N2 - Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described. Optimal therapy and outcomes in FGN presenting with crescentic GN is not currently known. We report an adult patient who presented with massive proteinuria and severe renal failure. The kidney biopsy revealed crescentic FGN (C-FGN). The patient remained dialysis dependent despite immunosuppressive therapy. We also briefly review FGN, and the few reported cases of C-FGN that presented as rapidly progressive or advanced renal failure in the literature.

AB - Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described. Optimal therapy and outcomes in FGN presenting with crescentic GN is not currently known. We report an adult patient who presented with massive proteinuria and severe renal failure. The kidney biopsy revealed crescentic FGN (C-FGN). The patient remained dialysis dependent despite immunosuppressive therapy. We also briefly review FGN, and the few reported cases of C-FGN that presented as rapidly progressive or advanced renal failure in the literature.

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