儿童期起病的系统性红斑狼疮的长期预后

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

作者信息

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

机构信息

Department of Pediatric Nephrology, Children Hospital, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.

Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, AP-HP, National Reference Centre for Rheumatic and Autoimmune Diseases in Children, RAISE, Paris and Lyon, France.

出版信息

Rheumatology (Oxford). 2025 Apr 1;64(4):2209-2213. doi: 10.1093/rheumatology/keae344.

DOI:10.1093/rheumatology/keae344

PMID:39008948

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.

摘要

目的

儿童期起病的系统性红斑狼疮（cSLE）患者的长期预后数据稀缺。本研究的目的是描述cSLE的长期预后，并确定与损伤发展和持续性疾病活动相关的因素。

方法

我们利用青少年炎性风湿病（JIR）队列数据库的PEDIALUP注册数据进行了一项回顾性多中心研究。在随访期间，从病历中收集人口统计学特征、临床表现、实验室、放射学、组织学和治疗数据。

结果

共纳入1971年至2015年间诊断的138例cSLE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损伤指数（DI）评分≥1，肌肉骨骼、皮肤、肾脏、神经和心血管损伤是最常见的表现。SLICC-DI评分≥1的患者比例随随访时间显著增加（P < 0.001）。多因素分析显示，随访时间与累积损伤风险增加相关（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。多因素分析显示，与白种人相比，撒哈拉以南非洲裔在最后一次就诊时患有活动性疾病的几率增加7倍（OR 7.44，95%CI 2.24，24.74，P = 0.0002）。