Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis

• Main Authors: H H Shah, J Thakkar, J M Pullman, A T Mathew
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2017-01-01
• Series: Indian Journal of Nephrology
• Subjects: Crescentic glomerulonephritis; fibrillary glomerulonephritis; glomerulonephritis; rapidly progressive glomerulonephritis
• Online Access: http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2017;volume=27;issue=2;spage=157;epage=160;aulast=Shah
• ISSN: 0971-4065; 1998-3662

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.