Clinicopathologic features in childhood-onset lupus nephritis with antineutrophil cytoplasmic antibody positivity——a multi-center retrospective study

Author:

Jin Bei1ORCID,Cheng Cheng1,Tan Meizhen2,Huang Jun34,Chen Lizhi1,Lin Zhilang1,Zeng Shuhan1,Yu Zihua34,Li Yingjie2,Jiang Xiaoyun1

Affiliation:

1. Department of Pediatric Rheumatology and Nephrology, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China

2. Department of Nephrology, Guangzhou Women and Children’s Medical Center, Guangzhou, China

3. Department of Nephrology, Rheumatology and Immunology, Fujian Children’s Hospital, Fuzhou, China

4. College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China

Abstract

Background Positive antineutrophil cytoplasmic antibody (ANCA) serology in adult-onset lupus nephritis (LN) is associated with more active disease and distinct renal pathology, but data with respect to childhood-onset LN remain scarce. Here, we aimed to determine the impact of positive ANCA serology on clinical and histopathologic features and renal outcomes in children with LN from multiple centers. Methods Clinical and histopathologic data of 61 ANCA-positive and 330 ANCA-negative LN children (1<age≤18 years) retrospectively enrolled from three pediatric nephrology centers were analyzed. Among them, 217 children were followed and survival analysis was performed. Results Among 61 ANCA-positive LN children, 86.9% of them had antimyeloperoxidase antibodies. Both ANCA-positive and ANCA-negative children had high disease activities with median SLEDAI-2K of 16 (13, 20). Hematuria was more prominent (urinary RBC +++ ∼ ++++: 45.9% vs 26.7%, p = 0.011), while fever (42.6% vs. 58.2%, p = 0.035), alopecia (3.3% vs. 14.5%, p = 0.019), photosensitivity (0% vs. 8.2%, p = 0.013), and pleurisy (4.9% vs. 15.8%, p = 0.026) were less common in ANCA-positive children. Higher proportions of segmental sclerosis (23.7% vs. 9.8%, p = 0.025), crescentic formation (36.4% vs. 16.3%, p = 0.009), and capillary wall thickening (24.5% vs. 11.0%, p = 0.01) were observed in biopsies of ANCA-positive children. Long-term renal survival did not differ significantly between two groups ( p = 0.300). Conclusions Positive ANCA serology in LN children was associated with different clinical and histopathologic features compared to those with negative ANCA serology. Further studies are needed to clarify the pathogenic role of ANCAs in childhood-onset LN and confirm their association with prognosis.

Funder

Sun Yat-Sen University Clinical Research 5010 Program

Clinical Research 2030 Project of the First Affiliated Hospital, Sun Yat-Sen University

Publisher

SAGE Publications

Subject

Rheumatology

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3