Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Clin J Am Soc Nephrol. 2018 Jan 6;13(1):63-72. doi: 10.2215/CJN.04290417. Epub 2017 Nov 21.


Background and objectives: Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients.

Design, setting, participants, & measurements: This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Their kidney biopsy samples were classified according to the histopathologic classification for ANCA-associated GN. Clinical data such as details of treatment were retrieved from clinical records. The primary outcome parameter was the occurrence of ESRD. Kidney survival was analyzed using the log-rank test and Cox regression analyses.

Results: The 5-year kidney survival rate was 34%, with an improved rate observed among patients diagnosed after 2007 (P=0.01). In patients with anti-GBM GN, histopathologic class and kidney survival were associated (P<0.001). Only one of 15 patients with a focal class biopsy sample (≥50% normal glomeruli) developed ESRD. Patients with a sclerotic class biopsy sample (≥50% globally sclerotic glomeruli) and patients with 100% cellular crescents did not recover from dialysis dependency at presentation. In multivariable analysis, dialysis dependency at presentation (hazard ratio [HR], 3.17; 95% confidence interval [95% CI], 1.59 to 6.32), percentage of normal glomeruli (HR, 0.97; 95% CI, 0.95 to 0.99), and extent of interstitial infiltrate (HR, 2.02; 95% CI, 1.17 to 3.50) were predictors of ESRD during follow-up.

Conclusions: Dialysis dependency, low percentage of normal glomeruli, and large extent of interstitial infiltrate are associated with poor kidney outcome in anti-GBM GN. Kidney outcome has improved during recent years; the success rate doubled after 2007.

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Keywords: ANCA; Antibodies, Antineutrophil Cytoplasmic; Autoantibodies; Biopsy; Confidence Intervals; Follow-Up Studies; Glomerular Basement Membrane; Goodpasture-s syndrome; Kidney Failure, Chronic; Multivariate Analysis; Regression Analysis; Retrospective Studies; Survival Rate; anti-GBM disease; antiglomerular basement membrane antibody; glomerulonephritis; kidney; kidney biopsy; renal dialysis.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Anti-Glomerular Basement Membrane Disease / diagnosis
  • Anti-Glomerular Basement Membrane Disease / immunology*
  • Anti-Glomerular Basement Membrane Disease / physiopathology
  • Anti-Glomerular Basement Membrane Disease / therapy
  • Autoantibodies / immunology*
  • Basement Membrane / immunology*
  • Biopsy
  • Disease Progression
  • Female
  • Humans
  • Kidney Failure, Chronic / immunology
  • Kidney Failure, Chronic / pathology
  • Kidney Failure, Chronic / physiopathology
  • Kidney Failure, Chronic / therapy
  • Kidney Glomerulus / immunology*
  • Kidney Glomerulus / pathology
  • Kidney Glomerulus / physiopathology
  • Male
  • Middle Aged
  • Prognosis
  • Renal Dialysis
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Young Adult


  • Autoantibodies
  • antiglomerular basement membrane antibody