Article Text

Download PDFPDF

622 Childhood-onset SLE Outcomes in the CARRA Lupus Registry
  1. Mary Beth Son1,
  2. Andrea Knight2,
  3. Laura Schanberg3,
  4. Emily von Scheven4,
  5. Shahla Amin5 and
  6. Aimee Hersh6
  1. 1Division of Immunology, Boston Children’s Hospital, Boston MA
  2. 2Division of Pediatric Rheumatology, Hospital for Sick Children, Toronto CA
  3. 3Duke Clinical Research Institute, Durham NC
  4. 4Division of Pediatric Rheumatology, UCSF Benioff Children’s Hospital, San Francisco CA
  5. 5Biostatistics of Childhood Arthritis and Rheumatology Research Alliance
  6. 6Division of Pediatric Rheumatology, University of Utah, Salt Lake City UT

Abstract

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.

Abstract 622 Table 1

Baseline Demographic Characteristics

Abstract 622 Table 2

Disease characteristics

Abstract 622 Figure 1

Achievement of LLDAS at each visit

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ .

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.