Exploring the prevalence of pulmonary involvement in juvenile-onset systemic lupus erythematosus: Data from the UK JSLE Cohort Study

IF 1.5 Q4 RHEUMATOLOGY

Reumatologia Clinica Pub Date : 2025-11-01 Epub Date: 2025-10-01 DOI:10.1016/j.reuma.2025.501975

Ayodele Faleye , Kamran Mahmood , Eslam Al-Abadi , Kate Armon , Kathryn Bailey , Mary Brennan , Coziana Ciurtin , Janet Gardner-Medwin , Kirsty Haslam , Daniel Hawley , Alice Leahy , Heather Rostron , Gulshan Malik , Zoe McLaren , Elena Moraitis , Athimalaipet Ramanan , Rangaraj Satyapal , Philip Riley , Ethan Sen , Alison Kinder , Eve Smith

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Abstract

Background

Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune disease with significant morbidity and mortality. Pulmonary manifestations in JSLE have not been comprehensively described in the literature to date.

Objectives

To report the frequency, clinical, and demographic characteristics of JSLE patients with pulmonary manifestations compared to those without.

Methods

United Kingdom (UK) JSLE Cohort Study participants aged < 18 years at diagnosis, with ≥4 American College of Rheumatology (ACR-1997) criteria for systemic lupus erythematosus (SLE), were eligible. Patients were grouped according to the presence or absence of pulmonary involvement. Pulmonary manifestations were described at diagnosis, 1-year, 2-year, and 5-year follow-up. Demographics and clinical characteristics of patients with/without pulmonary manifestations were compared.

Results

480 JSLE patients were included. Overall, 24.8% had pulmonary manifestations; 22.7% at diagnosis, 19.1% at 1 year, 17.2% at 2 years, and 22.4% patients at 5 years after diagnosis. Overall, the commonest manifestation was pulmonary serositis. Pulmonary involvement was associated with higher American College of Rheumatology (ACR)-1997 scores (p < 0.002) and higher pediatric version of British Isles Lupus Assessment Group (pBILAG) scores (p < 0.001) at diagnosis but there were no differences in Systemic Lupus International Collaborating Clinic Damage Index (SLICC-SDI) scores (p > 0.05). pBILAG defined pulmonary involvement was associated with increased frequency of constitutional (48.3 vs 26.1%), musculoskeletal (49.1 vs 26.1%), gastrointestinal (10.3 vs 3.8%), and hematological (37.9 vs 20.6%) involvement (all p < 0.05).

Conclusion

Pulmonary disease is common in JSLE. It is associated with wider organ involvement, suggesting a need for close monitoring and prompt treatment.

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探讨青少年发病系统性红斑狼疮肺部受累的患病率：来自英国JSLE队列研究的数据

背景：青少年发病的系统性红斑狼疮（JSLE）是一种罕见的自身免疫性疾病，发病率和死亡率都很高。迄今为止，JSLE的肺部表现尚未在文献中得到全面描述。目的对比无肺表现的JSLE患者，报告有肺表现的JSLE患者的发病频率、临床和人口学特征。方法：英国（UK） JSLE队列研究的参与者在诊断时年龄为18岁，具有≥4个美国风湿病学会（ACR-1997）系统性红斑狼疮（SLE）标准。患者根据有无肺部受累进行分组。在诊断时描述肺部表现，随访1年、2年和5年。比较有/无肺部症状患者的人口学特征和临床特征。结果纳入JSLE患者480例。总体而言，24.8%有肺部表现；诊断时22.7%，1年19.1%，2年17.2%，5年22.4%。总的来说，最常见的表现是肺浆液炎。肺部受损伤与诊断时较高的美国风湿病学会(ACR)-1997评分（p < 0.002）和较高的儿童版不列颠群岛狼疮评估组（pBILAG）评分（p < 0.001）相关，但系统性狼疮国际合作临床损害指数（SLICC-SDI）评分无差异（p > 0.05）。pBILAG定义的肺部受累与体格（48.3% vs 26.1%）、肌肉骨骼（49.1% vs 26.1%）、胃肠道（10.3 vs 3.8%）和血液学（37.9% vs 20.6%）受累的频率增加相关（均p <； 0.05）。结论JSLE多见于肺部疾病。它与更广泛的器官受累有关，提示需要密切监测和及时治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Reumatologia Clinica RHEUMATOLOGY-

CiteScore

2.40

自引率

6.70%

发文量

105

审稿时长

54 days

期刊介绍： Una gran revista para cubrir eficazmente las necesidades de conocimientos en una patología de etiología, expresividad clínica y tratamiento tan amplios. Además es La Publicación Oficial de la Sociedad Española de Reumatología y del Colegio Mexicano de Reumatología y está incluida en los más prestigiosos índices de referencia en medicina.