Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Abstract

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.

Podcast: This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2017_11_21_CJASNPodcast_18_1_v.mp3.

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.

Copyright © 2018 by the American Society of Nephrology.

Figures

Figure 1.

The kidney survival is highest in patients who were diagnosed between 2007 and 2015.

Figure 2.

The flowchart shows a favorable outcome in the focal class, a poor outcome in the sclerotic class, and variable outcomes in the mixed and crescentic class.

Figure 3.

Kidney survival is highest in patients with a focal class biopsy, and patient survival is similar in all histopathological classes. (A) Kidney survival according to histopathologic class. (B) Kidney survival according to percentage of normal glomeruli. (C) Patient survival according to histopathologic class.