Renal survival in children with glomerulonephritis with crescents: A pediatric nephrology research consortium cohort study

Abstract

There is no evidence-based definition for diagnosing crescentic glomerulonephritis. The prognostic implications of crescentic lesions on kidney biopsy have not been quantified. Our objective was to determine risk factors for end-stage kidney disease (ESKD) in patients with glomerulonephritis and crescents on kidney biopsy. A query of the Pediatric Nephrology Research Consortium’s Pediatric Glomerulonephritis with Crescents registry identified 305 patients from 15 centers. A retrospective cohort study was performed with ESKD as the primary outcome. Median age at biopsy was 11 years (range 1–21). The percentage of crescents was 3–100% (median 20%). Etiologies included IgA nephropathy (23%), lupus (21%), IgA vasculitis (19%) and ANCA-associated GN (13%), post-infectious GN (5%), and anti-glomerular basement membrane disease (3%). The prevalence of ESKD was 12% at one year and 16% at last follow-up (median = 3 years, range1–11). MediantimetoESKDwas100days. RiskfactorsforESKDincluded%crescents,presence of fibrous crescents, estimated GFR, and hypertension at biopsy. For each 1% increase in %crescents, therewasa3%decreaseinlogoddsof1-yearrenalsurvival(p = 0.003)anda2%decreaseinlogodds of renal survivalat last follow-up(p < 0.001). Thesefindings providean evidencebase for enrollment criteria for crescentic glomerulonephritis in future clinical trials.