Background/Purpose: Systemic autoimmune rheumatic diseases (SARD) include systemic lupus erythematous (SLE), idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc), Sjögren syndrome (SS), and systemic vasculitis (SV). Patients with childhood-onset SARD (ChildSARDs) develop disease during years of growth and organ maturation, which can result in unique influences on outcomes. We aim to assess disease outcomes of adults with ChildSARDs.

Methods: With an academic librarian, we developed a peer-reviewed search strategy using MEDLINE and Embase for English articles (1 January 1990 to 30 June 2023). We tested our searches against 26 known articles on adult outcomes in ChildSARDs to confirm and refine search coverage. Childhood-onset of disease was defined by authors ( < 14-18 years old). Studies with mixed populations (e.g. adolescent-adult, childhood, adult-onset) were excluded if: i) ChildSARDs adult outcomes were not reported separately, ii) studies primarily focused on non-clinical outcomes. Studies were graded for risk-of-bias using the Quality in Prognosis Studies (QuiPS) tool. Two reviewers independently graded studies before meeting to reach consensus. Any disputes were resolved by a third reviewer.

Results: The search identified 6848 papers, of which 40 met study criteria. No study meeting criteria was published before 2000, 4 (10%) between 2001-2010, 24 (60%) between 2011-2020, and 12 (30%) between 2021-2024. Most studies were conducted in Europe (43%) and North America (28%). 60% of studies were SLE, 25% IIM, 8% SSc, 5% SV, and 0% SS. 27 (68%) publications were cross-sectional in design. Commonly reported outcomes included disease activity (48%), accumulated damage (40%), cardiovascular (33%), and mortality (23%). Mean disease duration ranged from 5.5-23.9 years (N&#3f18), and median disease duration ranged from 8.08-29 years (N&#3f11). QuiPS identified a moderate to high risk-of-bias in the following domains: 95% of study participation, 92% of study attrition (N&#3f13, non-cross-sectional studies), 40% of prognostic factors, 70% of outcomes, 95% of confounding, 73% of statistical analysis.

Conclusion: There is growing interest in adult outcomes of ChildSARDs but data is still relatively scarce. The majority of studies demonstrated moderate to high risk-of-bias in multiple study domains. Researchers should commit to reporting outcomes using standard disease outcome measurements to help accumulate evidence. Investigators planning studies of adult outcomes of ChildSARDs should consider bias reduction measures during the design stage and acknowledge limitations where biases cannot be substantially reduced. Readers should be aware of design limitations when interpreting results from current studies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-scoping-review-of-outcomes-of-adults-with-childhood-onset-systemic-autoimmune-rheumatic-diseases/