Longitudinal disease- and steroid-related damage among adults with childhood-onset systemic lupus erythematosus

Abstract

Objectives: Determine whether adults with childhood-onset systemic lupus erythematosus (cSLE) are at increased risk for disease- and steroid-related damage as compared to individuals with adult-onset SLE (aSLE), and whether they continue to accumulate disease damage in adulthood.

Methods: Data derive from the 2007-2015 cycles of the Lupus Outcomes Study, a longitudinal cohort of adults with confirmed SLE. The Brief Index of Lupus Damage (BILD), a validated, patient-reported measure, was used to assess SLE-associated damage. Participants with baseline BILD were included (N = 1035). Diagnosis at age < 18 years was defined as cSLE (N = 113). Outcome variables included BILD score at baseline and follow-up, clinically significant change in BILD score over follow-up period, and presence of steroid-related damage (cataracts, osteoporosis-related fracture, avascular necrosis or diabetes mellitus).

Results: Mean time between baseline and follow up BILD assessment was 6.3 ± 1.7 years. In adjusted analyses, participants with cSLE and aSLE had similar levels of disease-related damage, and accumulated damage at similar rates. Participants with cSLE were more likely to report steroid-related damage (OR 1.7, 95% CI 1.1-2.8) in the adjusted analysis as compared to those with aSLE. Likelihood of steroid-related damage increased with disease duration for both groups, but was consistently higher among cSLE participants.

Conclusion: In this longitudinal cohort of adults with SLE, participants continued to accumulate damage at similar rates over time, regardless of age at onset or disease duration. Childhood-onset predicted increased risk of steroid-related damage. Aggressive use of steroid-sparing treatment strategies during childhood may be important to prevent steroid-related damage in adulthood.

Keywords: Adult-onset systemic lupus erythematosus; Brief Index of Lupus Damage (BILD) score; Childhood-onset systemic lupus erythematosus; Disease related damage; Steroid related damage.