Renal outcome and risk factors for end-stage renal disease in pediatric rapidly progressive glomerulonephritis.

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN), defined as acute nephritic syndrome with dramatic loss of renal function within a few months, is associated with crescentic glomerulonephritis (CresGN), which requires ≥50% crescents on pathology. The disease characteristics and renal outcome in children with RPGN, however, will differ according to the percentage of crescents.

METHODS: To evaluate the renal outcomes and factors associated with end-stage renal disease (ESRD), this retrospective cohort study assessed children aged ≤15 years with RPGN at a tertiary medical center.

RESULTS: Of 67 patients with RPGN, 32 (47.8%) were male; mean age was 10.6 ± 3.0 years; median follow up was 1.1 years (range, 0.02-9.17 years) and 24 (35.8%) progressed to ESRD. Post-infectious glomerulonephritis was the most frequent cause of RPGN (50.7%). The incidence of ESRD was significantly higher in patients with ≥50% than <50% crescents (57.9% vs 28.6%, P = 0.029). On Cox regression analysis, disease etiology, serum creatinine >3 mg/dL, need for acute dialysis, ≥80% crescents and ≥20% tubular atrophy and interstitial fibrosis (TA/IF) were associated with ESRD. On multivariate analysis, need for acute dialysis (HR, 2.8; 95% CI: 1.1-7.3, P = 0.041) and ≥20% TA/IF (HR, 4.8; 95% CI: 1.4-16.1, P = 0.011) were independent risk factors for the development of ESRD.

CONCLUSIONS: Approximately one-third of children with RPGN developed ESRD; and need for acute dialysis and TA/IF ≥20% were independent risk factors for ESRD.