OUTCOMES OF ADULTS WITH CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOUS: A SCOPING REVIEW

PV212 / #397 Poster Topic: AS23 - SLE-Diagnosis, Manifestations, & Outcomes Background/Purpose Systemic Lupus Erythematous (SLE) is a chronic, multisystem autoimmune disorder with a highly variable presentation and course. Approximately 20% of SLE cases are diagnosed within childhood. As increasing numbers of childhood-onset SLE (cSLE) patients are now seen surviving into adulthood, understanding their adulthood outcomes has become increasingly important. This scoping review aims to summarize the outcomes of adults with cSLE. Methods A peer-reviewed search strategy of OVID MEDLINE and Embase for English articles between 1 January 1990 to 30 June 2023 was developed with an academic librarian. The main search included all systemic autoimmune rheumatic diseases (SARD) and systemic vasculitides. This abstract focuses on only SLE studies. Our search was tested against 26 known SARD articles to verify search coverage. Childhood-onset of disease was as defined by authors (< 14-18 years old). Studies were excluded if: i) > 50% of the sample were not adults at final assessment and / or adult outcomes were not separately reported, iii) not a full-length article. Results from the search were uploaded into the Covidence website for subsequent workflow. Studies were graded for risk of bias using the Quality in Prognosis Studies (QuiPS) tool. Two reviewers independently graded the studies before meeting to reach consensus. Any disputes were resolved by a third reviewer. Results The total search identified 4379 papers, of which 24 SLE studies were included for this study. No study was published before 2000, 2 (8%) between 2001-2010, 16 (67%) between 2011-2020, and 6 (25%) between 2021-2024. Most studies were conducted in the United States (25%), Brazil (21%), and the Europe (13%). The vast majority of publications were cross-sectional in design (67%), followed by prospective cohorts (21%). Mean disease duration ranged from 5.5-23.9 years (13 studies) and median duration was 8.1-20 years (6 studies). Mean follow-up duration of longitudinal studies ranged from 6.3-14.2 years (4 studies) and median was 4 years (1 study). The most common reported outcomes were damage (50%), disease activity (38%) and mortality (21%). Other outcomes studied were depression, cognitive dysfunction, cardiovascular disease, malignancy, and clinical features. Mean SLICC damage index was 1.3-3.2 (4 studies) and median damage was 1-6 (5 studies), respectively. Mean and median SLE disease activity index [MM2] was 6.0-16 (4 studies) and median was 0-6 (5 studies), respectively. Mortality ranged from 2.5%-18.3% (5 studies). QuiPS identified a moderate to high risk-of-bias in the following domains: 92% of study participation, 100% of study attrition (N = 8, non-cross-sectional studies), 25% of prognostic factors, 71% of outcomes, 96% of confounding, 75% of statistical analysis. Conclusions There is increasing interest in the outcomes of cSLE adults as seen in the increasing number of publications. Current literature primarily focuses on disease damage, disease activity, and mortality. However, the studies showed significant biases, which perhaps reflects the difficulty of studying such a population. A collaborative and integrated approach between pediatric and adult rheumatologists to follow cSLE patients into adulthood, augmented by additional information sources such as population health data, will be essential to better understand the outcomes of cSLE patients with a wide spectrum of disease severity. Understanding current outcomes will inform better care for cSLE adults.

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