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Fibrillary glomerulonephritis

Author(s): Karthik P. Karanam | Brian Decker

Journal: Nephrology Reviews
ISSN 2035-8261

Volume: 4;
Issue: 2;
Date: 2012;
Original page

Keywords: fibrillary glomerulonephritis | membranoproliferative glomerulonephritis | Congo-red negative | nephrotic syndrome

Fibrillary glomerulonephritis (FGN) is a rare disorder characterized by Congo-red negative fibrillary deposits in the glomeruli. The incidence of FGN in native renal biopsies is only 0.8 to 1.5%. The pathological hallmark of FGN is fibrillary deposits in the mesangium and glomerular basement membrane. These fibrils are straight, non-branching and randomly oriented. In the majority of patients, FGN presents with the nephrotic syndrome. Renal insufficiency is present in two-thirds of patients with a mean serum creatinine of 2.1 mg/dL. Approximately one-half of FGN patients progress to end-stage renal disease (ESRD). Renal biopsy establishes the diagnosis of FGN. Light microscopy findings include amorphous acellular deposits in the glomeruli. In the mesangium these deposits have a smudged texture and form a pseudolinear or confluent granular pattern along the basement membrane. Immunofluorescent studies of the biopsy tissue in FGN shows staining for polyclonal IgG and complement with a preponderance for IgG and C3. Electron microscopy reveals extensive infiltration of the glomerular basement membrane with spike formation. Standard therapeutic treatments for FGN have not been well defined. The clinical outcome of patients with FGN is not encouraging. In a recent study 44% of patients with FGN progress to ESRD. The average time to progress to ESRD was 2 to 4 years. Immunosuppressant therapies that include corticosteroids, cyclophosphamide and cyclosporine and most recently rituximab have not demonstrated uniform clinical success.
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