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 CASE REPORT
Year : 2017  |  Volume : 27  |  Issue : 2  |  Page : 157-160

Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis


1 Department 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
2 Department of Pathology, Montefiore Medical Center, Bronx, New York, USA

Correspondence Address:
H H Shah
Hofstra Northwell School of Medicine, 100 Community Drive, 2nd Floor, Great Neck, New York 11021
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-4065.200521

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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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Indian Journal of Nephrology
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Online since 20th Sept '07