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. Inmultivariable 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.
Author supplied keywords
- ANCA
- Anti-GBM disease
- Antibodies antineutrophil
- Antiglomerular basement membrane antibody
- Autoantibodies
- Biopsy
- Confidence intervals
- Cytoplasmic
- Follow-Up studies
- Glomerular basement membrane
- Glomerulonephritis
- Goodpasture-s syndrome
- Kidney
- Kidney biopsy
- Kidney failure chronic
- Multivariate analysis
- Regression analysis
- Renal dialysis
- Retrospective studies
- Survival rate
Cite
CITATION STYLE
van Daalen, E. E., Jennette, J. C., McAdoo, S. P., Pusey, C. D., Alba, M. A., Poulton, C. J., … Bajema, I. M. (2018). Predicting outcome in patients with anti-GBM glomerulonephritis. Clinical Journal of the American Society of Nephrology, 13(1), 63–72. https://doi.org/10.2215/CJN.04290417
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