Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

<jats:sec> <jats:title>Background and objectives</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Design, setting, participants, & measurements</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>The 5-year kidney survival rate was 34%, with an improved rate observed among patients diagnosed after 2007 (<jats:italic toggle="yes">P</jats:italic>=0.01). In patients with anti-GBM GN, histopathologic class and kidney survival were associated (<jats:italic toggle="yes">P</jats:italic><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.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Podcast</jats:title> <jats:p>This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2017_11_21_CJASNPodcast_18_1_v.mp3 </jats:p> </jats:sec>