Long-term clinical outcomes in a cohort of adults with childhood-onset Systemic Lupus Erythematosus.
Schlüsselwörter
Abstrakt
OBJECTIVE
Childhood-onset SLE (cSLE) is a severe lifelong multisystem autoimmune disease. Long-term outcome data are limited. Here, we report clinical characteristics and health-related quality of life (HRQOL) of adults with cSLE.
METHODS
Patients underwent a single study visit comprising a structured history and physical examination. Disease activity (SLEDAI-2K), damage (SLICC-Damage Index (SDI)) and HRQOL (SF-36) were determined. Medical records were retrieved.
RESULTS
In total, 111 cSLE patients were included, median disease duration 20 years, 91% female and 72% white. Disease activity was low (median SLEDAI 4), 71% of patients used prednisone, hydroxychloroquine and/or other disease-modifying anti-rheumatic drugs. The vast majority of new cSLE-related manifestations developed within 2 years of diagnosis. Damage like myocardial infarctions started occurring after 5 years. Most patients (62%) had damage, predominantly in the musculoskeletal, neuropsychiatric and renal systems. Cerebrovascular accidents, renal transplants, replacement arthroplasties and myocardial infarctions, developed at young age (median age 20, 24, 34 and 39 years respectively). Multivariate logistic regression showed that damage accrual was associated with disease duration (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). HRQOL was impaired compared to the Dutch population. Presence of damage reduced HRQOL in one domain. High disease activity (SLEDAI≥8) and changes in physical appearance strongly reduced HRQOL (4/8 and 7/8 domains).
CONCLUSIONS
The majority of adults with cSLE in this large cohort developed significant damage at young age and have impaired HRQOL without achieving drug free remission, illustrating the great impact of cSLE on future life. This article is protected by copyright. All rights reserved.
