title: Fibrillary glomerulonephritis: a report of 66 cases from a single institution.
abstract: OBJECTIVE
  Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease. Most previously reported cases were idiopathic. To better define the clinical-pathologic spectrum and prognosis, we report the largest single-center series with the longest follow-up.
  
  
  METHODS
  The characteristics of 66 FGN patients who were seen at Mayo Clinic, Rochester, between 1993 and 2010 are provided.
  
  
  RESULTS
  The mean age at diagnosis was 53 years. Ninety-five percent of patients were white, and the female:male ratio was 1.2:1. Underlying malignancy (most commonly carcinoma), dysproteinemia, or autoimmune disease (most commonly Crohn's disease, SLE, Graves' disease, and idiopathic thrombocytopenic purpura), were present in 23, 17, and 15% of patients, respectively. Presentation included proteinuria (100%), nephrotic syndrome (38%), renal insufficiency (66%), hematuria (52%), and hypertension (71%). The most common histologic pattern was mesangial proliferative/sclerosing GN followed by membranoproliferative GN. During an average of 52.3 months of follow-up for 61 patients with available data, 13% had complete or partial remission, 43% had persistent renal dysfunction, and 44% progressed to ESRD. The disease recurred in 36% of 14 patients who received a kidney transplant. Independent predictors of ESRD by multivariate analysis were older age, higher creatinine and proteinuria at biopsy, and higher percentage of global glomerulosclerosis.
  
  
  CONCLUSIONS
  Underlying malignancy, dysproteinemia, or autoimmune diseases are not uncommon in patients with FGN. Prognosis is poor, although remission may occur in a minority of patients without immunosuppressive therapy. Age, degree of renal impairment at diagnosis, and degree of glomerular scarring are predictors of renal survival.
authors: Mayo Clinic, Division of Anatomic Pathology, Hilton 10-20, 200 First Street SW, Rochester, MN 55905, USA. nasr.samih@mayo.edu
authors: Samih H
authors: SH
authors: Nasr
authors: Anthony M
authors: AM
authors: Valeri
authors: Lynn D
authors: LD
authors: Cornell
authors: Mary E
authors: ME
authors: Fidler
authors: Sanjeev
authors: S
authors: Sethi
authors: Nelson
authors: N
authors: Leung
authors: Fernando C
authors: FC
authors: Fervenza
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001797:Blood Protein Electrophoresis; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D016016:Proportional Hazards Models