Validation of a renal risk score in a cohort of children with ANCA-associated glomerulonephritis.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is extremely rare in children. Renal involvement is a common and severe complication of AAV as it can cause end stage kidney disease (ESKD). ANCA renal risk score (ARRS) is helpful in predicting long-term ESKD in patients with ANCA-associated glomerulonephritis (AAGN). This retrospective study included 61 consecutive patients with kidney biopsy specimen-proven AAGN from Clinical Center for Children's Kidney Disease in China. Each patient was assessed by eGFR, normal glomeruli, and tubular atrophy/interstitial fibrosis, and the renal outcome was evaluated using the ARRS. Based on the ARRS, 27 (44.26%), 21 (34.43%), and 13 (21.31%) patients were divided into the low-risk, medium-risk, and high-risk groups, respectively. The median follow-up period was 46.36 (14.58-95.62) months. The high-risk group had worse renal outcomes than the low-risk group (p< 0.05) and the medium-risk group (p < 0.05). COX multivariate regression analysis showed that eGFR ≤ 15 ml/min/1.73 m (p = 0.015, Hazard Ratio (HR) = 9.574, 95% CI 4.205-25.187) and ARRS (p = 0.012, HR = 2.115, 95% CI 1.206-4.174) were independent risk factors for ESKD.The area under the curve for ESKD prediction of ARRS was 0.880, and the best cutoff value was 5.50. Delong test result showed that ARRS exhibited better predictive value for ESKD than the Berden classification (p < 0.001) and rapidly progressive glomerulonephritis (p < 0.001). This is the first study to investigate the value of the ARRS for predicting renal prognosis among Chinese children. The ARRS is a preferred index that can predict ESKD in Chinese children with AAGN.