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

Li-Wen Tan ¹Jun-Li Wan ¹Chun-Hua Zhu ²Hong Xu ³Zheng-Kun Xia ⁴Li-Zhi Chen ⁵Xiao-Chuan Wu ⁶Fang Wang ⁷Xiao-Rong Liu ⁸Cheng-Guang Zhao ⁹Xiao-Zhong Li ¹⁰Jian-Hua Mao ¹¹Xiao-Wen Wang ¹²Wen-Yan Huang ¹³Yu-Hong Li ¹⁴Jian-Jiang Zhang ¹⁵Shi-Pin Feng ¹⁶Jun Yang ¹⁷Jiao-Jiao Liu ³Chun-Lin Gao ⁴Li-Ping Rong ⁵Lan-Jun Shuai ⁶Ke Xu ⁷He-Jia Zhang ⁸Qiu Li ¹Ai-Hua Zhang ²Mo Wang¹

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作者信息

1. Department of Nephrology,Children's Hospital of Chongqing Medical University,Zhongshan 2nd Rd.136,Chongqing 400014,China;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;Chongqing Key Laboratory of Pediatrics,Children's Hospital of Chongqing Medical Un
2. Department of Nephrology,Children's Hospital of Nanjing Medical University,72 Guangzhou Road,Nanjing 210008,China
3. Department of Nephrology,Children's Hospital of Fudan University,National Paediatric Medical Center of China,Shanghai,China
4. Department of Pediatrics,Jinling Hospital,Nanjing Medical University,Nanjing,China;Department of Pediatrics,Jinling Hospital,The First School of Clinical Medicine,Southern Medical University,Nanjing,China;Department of Pediatrics,Jinling Hospital,Medical School of Nanjing University,Nanjing,China
5. Department of Pediatric Nephrology and Rheumatology,The First Affiliated Hospital of Sun Yat-Sen University,Guangzhou,China
6. Department of Pediatrics,The Second Xiangya Hospital of Central South University,Changsha,China
7. Department of Pediatrics,Peking University First Hospital,Beijing,China
8. Department of Nephrology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;National Center for Children's Health,Beijing,China
9. Department of Pediatrics,Shengjing Hospital of China Medical University,Shenyang,China
10. Department of Nephrology and Immunology,Children's Hospital of Soochow University,Suzhou,China
11. Department of Nephrology,Children Hospital,Zhejiang University School of Medicine,Hangzhou,China
12. Department of Nephrology,Wuhan Children's Hospital(Wuhan Maternal and Child Healthcare Hospital),Tongji Medical College,Huazhong University of Science &Technology,Wuhan,China
13. Department of Nephrology and Rheumatology,Shanghai Children's Hospital,School of medicine,Shanghai Jiao Tong University,Shanghai,China
14. Pediatric Nephrology Department,Guiyang Maternal &Child Health Care Hospital,Guiyang,China
15. Department of Pediatrics,The First Affiliated Hospital of Zhengzhou University,Zhengzhou,China
16. Department of Nephrology,Chengdu Women and Children Central Hospital,Chengdu 610041,China
17. Department of Rheumatology and Immunology,Shenzhen Children's Hospital,Shenzhen,China
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Abstract

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.

Key words

Antineutrophil cytoplasmic antibody/End-stage renal disease/Glomerulonephritis/Pediatric nephrology/Vasculitis

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基金项目

国家重点研发计划(2021YFC2702002)

国家重点研发计划(2022YFC2705100)

Key Research and Development Projects of Jiangsu Province(BE2021607)

National Clinical Research Center for Child Health and Disorders(NCRCCHD-2020-GP-01)

出版年

2024

世界儿科杂志(英文版)

CSTPCD

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参考文献量25

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