摘要Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vas-culitis featured by liability to renal involvement.However,there are few studies on the risk factors and predictive models for renal outcomes of AAV in children.Methods Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospec-tively.The risk factors and predictive model of end-stage renal disease(ESRD)in AAV were explored.Results Renal involvement was the most typical manifestation(95.5％),and the crescent was the predominant pathological lesion(84.9％).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6％developed ESRD,and the median time to ESRD was 3.20 months.The eGFR[P=0.006,odds ratio(OR)=0.955,95％confidence inter-val(CI)=0.924-0.987]and the percentages of global glomerulosclerosis(pGGS;P=0.018,OR=1.060,95％CI=1.010-1.112)were independent risk factors for ESRD of renal biopsy.Based on the pGGS and eGFR at renal biopsy,we developed three risk grades of ESRD and one predictive model.The Kaplan-Meier curve indicated that renal outcomes were signifi-cantly different in different risk grades(P＜0.001).Compared with serum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,P＜0.001)and a lower coefficient of variation(0.07 versus 0.92)in external validation.Conclusions Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into ESRD.The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children.