Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis:a nationwide retrospective study in China  

作　　者：Li-Wen Tan Jun-Li Wan Chun-Hua Zhu Hong Xu Zheng-Kun Xia Li-Zhi Chen Xiao-Chuan Wu Fang Wang Xiao-Rong Liu Cheng-Guang Zhao Xiao-Zhong Li Jian-Hua Mao Xiao-Wen Wang Wen-Yan Huang Yu-Hong Li Jian-Jiang Zhang Shi-Pin Feng Jun Yang Jiao-Jiao Liu Chun-Lin Gao Li-Ping Rong Lan-Jun Shuai Ke Xu He-Jia Zhang Qiu Li Ai-Hua Zhang Mo Wang 

机构地区：[1]Department of Nephrology,Children’s Hospital of Chongqing Medical University,Zhongshan 2nd Rd.136,Chongqing,400014,China [2]Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Children’s Hospital of Chongqing Medical University,Chongqing,China [3]Chongqing Key Laboratory of Pediatrics,Children’s Hospital of Chongqing Medical University,Chongqing,China [4]Department of Nephrology,Children’s Hospital of Nanjing Medical University,72 Guangzhou Road,Nanjing,210008,China [5]Department of Nephrology,Children’s Hospital of Fudan University,National Paediatric Medical Center of China,Shanghai,China [6]Department of Pediatrics,Jinling Hospital,Nanjing Medical University,Nanjing,China [7]Department of Pediatrics,Jinling Hospital,The First School of Clinical Medicine,Southern Medical University,Nanjing,China [8]Department of Pediatrics,Jinling Hospital,Medical School of Nanjing University,Nanjing,China [9]Department of Pediatric Nephrology and Rheumatology,The First Affiliated Hospital of Sun Yat-Sen University,Guangzhou,China [10]Department of Pediatrics,The Second Xiangya Hospital of Central South University,Changsha,China [11]Department of Pediatrics,Peking University First Hospital,Beijing,China [12]Department of Nephrology,Beijing Children’s Hospital,Capital Medical University,Beijing,100045,China [13]National Center for Children’s Health,Beijing,China [14]Department of Pediatrics,Shengjing Hospital of China Medical University,Shenyang,China [15]Department of Nephrology and Immunology,Children’s Hospital of Soochow University,Suzhou,China [16]Department of Nephrology,Children Hospital,Zhejiang University School of Medicine,Hangzhou,China [17]Department of Nephrology,Wuhan Children’s Hospital(Wuhan Maternal and Child Healthcare Hospital),Tongji Medical College,Huazhong University of Science&Technology,Wuhan,China

出　　处：《World Journal of Pediatrics》2024年第5期506-516,共11页世界儿科杂志（英文版）

基　　金：approved by the Ethics Committee of the Children’s Hospital of Chongqing Medical University(approval Number:149/2022);other enrolled centers.This study was registered at the Chinese Clinical Trial Registry(registered number:ChiCTR2000034203).

摘　　要：Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis 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 retrospectively.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 interval(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 significantly 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.

关 键 词：Antineutrophil cytoplasmic antibody End-stage renal disease GLOMERULONEPHRITIS Pediatric nephrology VASCULITIS 

分 类 号：R725.8[医药卫生—儿科]