Exploring the prevalence of pulmonary involvement in juvenile-onset systemic lupus erythematosus: Data from the UK JSLE Cohort Study
Year: 2025
Type: article
Study design: Observational Study
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.
Authors: 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, Nick Wilkinson, Christian Hedrich, Michael Beresford, Eve Smith
Institutions: Alder Hey Children's NHS Foundation Trust, Alder Hey Children's Hospital, Birmingham Children's Hospital, University of Cambridge, Cambridge University Hospitals NHS Foundation Trust, Oxford University Hospitals NHS Trust, Royal Hospital for Sick Children, University College London, University of Glasgow, Bradford Royal Infirmary, Sheffield Children's NHS Foundation Trust, Sheffield Children's Hospital, Southampton General Hospital, Leeds Teaching Hospitals NHS Trust, Royal Aberdeen Children's Hospital, Liverpool University Hospitals NHS Foundation Trust, Great Ormond Street Hospital, University Hospitals Bristol NHS Foundation Trust, University of Bristol, Nottingham University Hospitals NHS Trust, Royal Manchester Children's Hospital, Great North Children's Hospital, Newcastle University, University Hospitals of Leicester NHS Trust, Guy's and St Thomas' NHS Foundation Trust, Evelina London Children's Healthcare, University of Liverpool
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,170
OpenAlex ID: W4416224933