Background/Purpose: Cognitive dysfunction (including executive dysfunction) affects up to 60% of youth with childhood-onset systemic lupus erythematosus (cSLE), with potential adverse effects on health-related quality of life, and medication adherence. Patients with cSLE are often diagnosed during a critical period of neurodevelopment, and changes in executive function (EF) over time have not been well-studied in cSLE compared to typically developing children. We aimed to characterize the course of EF over time in cSLE patients matched to healthy controls.

Methods: We examined a prospective longitudinal sample of patients with cSLE (ages 12-17, met ACR or SLICC classification criteria for SLE), along with age and sex-matched healthy controls over two time points (baseline and 1 year later) collected from January 2020 to May 2023. Participants completed the self-report Behavior Rating Inventory of Executive Function 2 (BRIEF-2) that generated overall EF difficulties index score (global executive composite (GEC)), and three sub-indices – behavioral regulation index (BRI), cognitive regulation index (CRI), and emotion regulation index (ERI)). T-scores are transformations of raw scores based on age and sex norms. A higher T-score indicates a greater degree of difficulty in executive functioning. Disease measures including SLE Disease Activity Index 2000 (SLEDAI-2K), SLICC/ACR damage index (SDI), glucocorticoid use at visit and cumulative use, and disease duration were collected. The primary outcome was EF measured by GEC, BRI, CRI, and ERI T-scores, with a focus on GEC. T-tests were performed to examine within-group and between-group comparisons of mean scores at baseline and 1-year, and of change in mean scores (1 year minus baseline) respectively, for cSLE participants and controls.

Results: Twenty-two patients with cSLE and 10 controls were included. For cSLE, the mean age was 15.7 ± SD 1.5 years, 86% were female, median SLEDAI-2K score was 2 (IQR 2-4) and median disease duration was 2 years (IQR 1.2-4.3) (Table1). In cSLE patients compared to controls, mildly elevated GEC T-scores were present in 18% (n=4) vs. 1 (10%); potentially and/or clinically elevated GEC T-scores were present in 41% (n=9) vs 30 %(n=3) (Table2) at baseline or 1-year timepoints. While the mean CRI T-score showed a small improvement from baseline and 1 year later (60 vs 56.1), there were no statistically significant differences for within-group comparisons of any of the BRIEF-T scores over time for both cSLE and controls (Table 2). There were also no significant differences between the cSLE and control groups for mean change in the BRIEF-T scores over time (GEC, t-value=0.78, p=0.43; BRI t-value= -0.28, p=0.77, CRI t-value= -1.2, p=0.20, ERI t-value 0.99, p=0.32).

Conclusion: Self-reported EF was stable over a one-year period in this small cohort of patients with cSLE and controls. Although no significant differences in mean scores for EF were found between cSLE and controls, 59% (versus 40% of HC) had at least mild difficulties in EF. More longitudinal research is needed with longer follow up to identify cognitive function trajectories and at-risk groups for executive dysfunction in cSLE.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/longitudinal-assessment-of-self-reported-executive-function-in-youth-with-childhood-onset-lupus/