Long-Term Clinical Outcomes in a Cohort of Adults With Childhood-Onset Systemic Lupus Erythematosus
Objective Childhood‐onset systemic lupus erythematosus (SLE) is a severe, lifelong, multisystem autoimmune disease. Long‐term outcome data are limited. This study was undertaken to identify clinical characteristics and health‐related quality of life (HRQoL) of adults with childhood‐onset SLE. Methods Patients participated in a single study visit comprising a structured history and physical examination. Disease activity (scored using the SLE Disease Activity Index 2000 [SLEDAI‐2K]), damage (scored using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]), and HRQoL (scored using the Short Form 36 Health Survey) were assessed. Medical records were reviewed. Results In total, 111 childhood‐onset SLE patients were included; the median disease duration was 20 years, 91% of patients were female, and 72% were white. Disease activity was low (median SLEDAI‐2K score 4), and 71% of patients received prednisone, hydroxychloroquine (HCQ), and/or other disease‐modifying antirheumatic drugs. The vast majority of new childhood‐onset SLE–related manifestations developed within 2 years of diagnosis. Damage such as myocardial infarctions began occurring after 5 years. Most patients (62%) experienced damage, predominantly in the musculoskeletal, neuropsychiatric, and renal systems. Cerebrovascular accidents, renal transplants, replacement arthroplasties, and myocardial infarctions typically occurred at a young age (median age 20 years, 24 years, 34 years, and 39 years, respectively). Multivariate logistic regression analysis showed that damage accrual was associated with disease duration (odds ratio [OR] 1.15, P < 0.001), antiphospholipid antibody positivity (OR 3.56, P = 0.026), and hypertension (OR 3.21, P = 0.043). Current HCQ monotherapy was associated with an SDI score of 0 (OR 0.16, P = 0.009). In this cohort, HRQoL was impaired compared to the overall Dutch population. The presence of damage reduced HRQoL scores in 1 domain. High disease activity (SLEDAI‐2K score ≥8) and changes in physical appearance strongly reduced HRQoL scores (in 4 of 8 domains and 7 of 8 domains, respectively). Conclusion The majority of adults with childhood‐onset SLE in this large cohort developed significant damage at a young age and had impaired HRQoL without achieving drug‐free remission, illustrating the substantial impact of childhood‐onset SLE on future life.
|Persistent URL||dx.doi.org/10.1002/art.40697, hdl.handle.net/1765/115254|
|Journal||ARTHRITIS & RHEUMATOLOGY|
de Groot, N.M.S, Shaikhani, D., Teng, Y.K.O, de Leeuw, K, Bijl, M.A, Dolhain, R.J.E.M, … Kamphuis, S.S.M. (2019). Long-Term Clinical Outcomes in a Cohort of Adults With Childhood-Onset Systemic Lupus Erythematosus. ARTHRITIS & RHEUMATOLOGY, 71(2), 290–301. doi:10.1002/art.40697