Long-Term Clinical Outcomes in a Cohort of Adults With Childhood-Onset Systemic Lupus Erythematosus

Arthritis Rheumatol. 2019 Feb;71(2):290-301. doi: 10.1002/art.40697.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Antibodies, Antiphospholipid / immunology
  • Antirheumatic Agents / therapeutic use*
  • Child
  • Child, Preschool
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Hypertension / epidemiology
  • Kidney Transplantation / statistics & numerical data
  • Logistic Models
  • Lupus Erythematosus, Systemic / drug therapy*
  • Lupus Erythematosus, Systemic / epidemiology
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Nephritis / epidemiology
  • Lupus Nephritis / surgery
  • Lupus Vasculitis, Central Nervous System / epidemiology
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Musculoskeletal Diseases / epidemiology
  • Myocardial Infarction / epidemiology
  • Netherlands / epidemiology
  • Odds Ratio
  • Prednisone / therapeutic use
  • Quality of Life*
  • Severity of Illness Index
  • Stroke / epidemiology
  • Young Adult


  • Antibodies, Antiphospholipid
  • Antirheumatic Agents
  • Glucocorticoids
  • Hydroxychloroquine
  • Prednisone