Objective This study aims to describe the demographic features, cumulative clinical manifestations, and treatments in a large childhood-onset (cSLE) cohort in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. This study also assesses lupus low disease activity state (LLDAS) and examines predictors of the first attainment of LLDAS.
Methods We performed a retrospective cohort study of patients with cSLE enrolled in the CARRA Registry between March 2017 to December 2021. Inclusion criteria included: 1) diagnosis of cSLE at <18 years based on Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) criteria; 2) enrollment within two years of cSLE diagnosis or at the time of a flare of lupus nephritis (LN); and 3) enrollment prior to 21 years of age. Sociodemographic and clinical data were summarized using descriptive statistics. A chi-squared test was used to assess the association between LLDAS and the categorical variables. We used logistic regression to assess enrollment predictors of LLDAS attainment at 6, 12 and 18 months of follow-up
Results The CARRA Registry includes 779 patients with cSLE (table 1). In this ethnically and racially diverse cohort, the median SLEDAI at enrollment was 4 (IQR 2-10) and median time from enrollment to end of follow up was 22 (IQR 11 to 33) months. At enrollment, 18% of patients had a SLICC damage index score greater than zero (table 2). At the end of the follow-up period, almost 50% of patients developed lupus nephritis. 5.7% and 12.4% had neurological manifestations per ACR and SLICC criteria, respectively. 94.6% were prescribed hydroxychloroquine and participants received a variety of immunosuppressive therapies. The percentage of visits where LLDAS was achieved is shown in figure 1. In multivariate analysis, statistically significant predictors of LLDAS attainment included time from diagnosis and 6, 12 and 18 months of follow-up and baseline hydroxychloroquine use.
Conclusion The CARRA Registry has enrolled a large, racially and ethnically diverse cohort of cSLE patients that are early in their disease course, exhibit moderate disease activity and have minimal damage scores. The use of hydroxychloroquine in this cohort is high; hydroxychloroquine use at enrollment was a strong predictor of LLDAS attainment. This cohort provides a unique opportunity to study longitudinally the impact of disease activity and immunosuppressive medications in a young SLE cohort.
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