title: Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis.
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.
authors.affiliation: 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.
authors.forename: H H
authors.initials: HH
authors.lastname: Shah
authors.affiliation: 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.
authors.forename: J
authors.initials: J
authors.lastname: Thakkar
authors.affiliation: Department of Pathology, Montefiore Medical Center, Bronx, New York, USA.
authors.forename: J M
authors.initials: JM
authors.lastname: Pullman
authors.affiliation: 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.
authors.forename: A T
authors.initials: AT
authors.lastname: Mathew
keywords: Crescentic glomerulonephritis; fibrillary glomerulonephritis; glomerulonephritis; rapidly progressive glomerulonephritis