Long-term outcomes of childhood-onset systemic lupus erythematosus.

IF 4.7 2区医学 Q1 RHEUMATOLOGY

Rheumatology Pub Date : 2025-04-01 DOI:10.1093/rheumatology/keae344

Anne Mirguet, Florence A Aeschlimann, Irene Lemelle, Roland Jaussaud, Paul Decker, Thomas Moulinet, Shirine Mohamed, Pierre Quartier, Michael Hofer, Olivia Boyer, Alexandre Belot, Aurélie Hummel, Nathalie Costedoat-Chalumeau, Brigitte Bader-Meunier

{"title":"Long-term outcomes of childhood-onset systemic lupus erythematosus.","authors":"Anne Mirguet, Florence A Aeschlimann, Irene Lemelle, Roland Jaussaud, Paul Decker, Thomas Moulinet, Shirine Mohamed, Pierre Quartier, Michael Hofer, Olivia Boyer, Alexandre Belot, Aurélie Hummel, Nathalie Costedoat-Chalumeau, Brigitte Bader-Meunier","doi":"10.1093/rheumatology/keae344","DOIUrl":null,"url":null,"abstract":"Objective: Data on the long-term outcome of patients with childhood-onset SLE (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persistent disease activity.Methods: We conducted a retrospective multicentre study using data from the PEDIALUP registry of the Juvenile Inflammatory Rheumatism (JIR) cohort database. Demographic characteristics, clinical manifestations, laboratory, radiological, histological and treatment data were collected from medical records during follow-up.Results: A total of 138 patients with cSLE, diagnosed between 1971 and 2015, were included. With a median follow-up of 15.4 [9.6-22.4] years, 51% of patients had a SLICC-damage index (DI) score ≥1 at last follow-up with the musculoskeletal, cutaneous, renal, neurological and cardiovascular damage being the most common manifestations. The proportion of patients with a SLICC-DI score ≥1 increased significantly with the duration of the follow-up (P < 0.001). On multivariate analysis, duration of follow-up was associated with increased risk of cumulative damage (OR 1.08, 95% CI 1.01, 1.15, P = 0.035). At the last visit, 34% of patients still had active disease with a SLEDAI score of ≥6. On multivariate analysis, sub-Saharan African ethnicity was associated with 7-fold increased odds of having active disease at the last visit compared with Caucasians (OR 7.44, 95% CI 2.24, 24.74, P = 0.0002).Conclusion: The prevalence of damage remains high in patients with cSLE even when the diagnosis of cSLE has been made in the recent decades.","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2209-2213"},"PeriodicalIF":4.7000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/rheumatology/keae344","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Objective: Data on the long-term outcome of patients with childhood-onset SLE (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persistent disease activity.

Methods: We conducted a retrospective multicentre study using data from the PEDIALUP registry of the Juvenile Inflammatory Rheumatism (JIR) cohort database. Demographic characteristics, clinical manifestations, laboratory, radiological, histological and treatment data were collected from medical records during follow-up.

Results: A total of 138 patients with cSLE, diagnosed between 1971 and 2015, were included. With a median follow-up of 15.4 [9.6-22.4] years, 51% of patients had a SLICC-damage index (DI) score ≥1 at last follow-up with the musculoskeletal, cutaneous, renal, neurological and cardiovascular damage being the most common manifestations. The proportion of patients with a SLICC-DI score ≥1 increased significantly with the duration of the follow-up (P < 0.001). On multivariate analysis, duration of follow-up was associated with increased risk of cumulative damage (OR 1.08, 95% CI 1.01, 1.15, P = 0.035). At the last visit, 34% of patients still had active disease with a SLEDAI score of ≥6. On multivariate analysis, sub-Saharan African ethnicity was associated with 7-fold increased odds of having active disease at the last visit compared with Caucasians (OR 7.44, 95% CI 2.24, 24.74, P = 0.0002).

Conclusion: The prevalence of damage remains high in patients with cSLE even when the diagnosis of cSLE has been made in the recent decades.

查看原文本刊更多论文

儿童期系统性红斑狼疮的长期预后。

目的：有关儿童期系统性红斑狼疮（cSLE）患者长期预后的数据很少。本研究旨在描述系统性红斑狼疮的长期预后，并确定与损害的发展和持续性疾病活动相关的因素：我们利用青少年炎症性风湿病（JIR）队列数据库的 PEDIALUP 登记数据进行了一项回顾性多中心研究。研究人员从随访期间的医疗记录中收集了人口统计学特征、临床表现、实验室、放射学、组织学和治疗数据：结果：共纳入了138名在1971年至2015年间确诊的系统性红斑狼疮患者。中位随访时间为15.4[9.6-22.4]年，51%的患者在最后一次随访时SLICC-损害指数评分≥1，肌肉骨骼、皮肤、肾脏、神经和心血管损害是最常见的表现。随着随访时间的延长，SLICC-DI评分≥1分的患者比例明显增加（p< 0.001）。多变量分析显示，随访时间与累积损害风险的增加有关（OR 1.08，95% CI 1.01，1.15，p= 0.035）。在最后一次就诊时，34% 的患者仍有活动性疾病，SLEDAI 评分≥ 6。多变量分析显示，与白种人相比，撒哈拉以南非洲裔患者在最后一次就诊时患活动性疾病的几率增加了 7 倍（OR 7.44，95% CI 2.24，24.74，p= 0.0002）：结论：即使近几十年来已确诊为 c-SLE，但损伤在 c-SLE 患者中的发生率仍然很高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Rheumatology 医学-风湿病学

CiteScore

9.40

自引率

7.30%

发文量

1091

审稿时长

2 months

期刊介绍： Rheumatology strives to support research and discovery by publishing the highest quality original scientific papers with a focus on basic, clinical and translational research. The journal’s subject areas cover a wide range of paediatric and adult rheumatological conditions from an international perspective. It is an official journal of the British Society for Rheumatology, published by Oxford University Press. Rheumatology publishes original articles, reviews, editorials, guidelines, concise reports, meta-analyses, original case reports, clinical vignettes, letters and matters arising from published material. The journal takes pride in serving the global rheumatology community, with a focus on high societal impact in the form of podcasts, videos and extended social media presence, and utilizing metrics such as Altmetric. Keep up to date by following the journal on Twitter @RheumJnl.