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

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.