A Scoping Review of Outcomes and the Quality of Studies of Adults with Childhood-Onset Systemic Autoimmune Rheumatic Diseases
Bibliographic record
Abstract
Objectives To assess reported outcomes of adults with childhood-onset systemic autoimmune rheumatic diseases (ChildSARDs), including systemic lupus erythematous (SLE), idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc), Sjögren syndrome (SjS) and systemic vasculitis (SV). Methods With a 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 ChildSARD adult outcomes to verify search coverage. Childhood-onset of disease was defined by authors (<14-18 years old). Studies with mixed populations (eg, adolescent-adult, childhood, adult onset) were excluded if: i) >50% of the sample were still children at final assessment, ii) ChildSARD adult outcomes were not reported separately. 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 (LL). 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 (33%) and the United States (28%). 60% of studies were in SLE, 25% IIM, 8% SSc, 5% SV, and 0% SjS. The majority of publications were cross-sectional in design (68%). Mean disease duration ranged from 5.5-23.9 years. Mean follow-up duration of longitudinal studies ranged from 7.6-14.2 years. The most common reported outcomes were accumulated damage (25%), mortality (23%), and disease activity (18%). QuiPS identified a moderate to high risk-of-bias in the following domains: 95% of study participation, 92% of study attrition (N=13, non-cross-sectional studies), 40% of prognostic factors, 70% of outcomes, 95% of confounding, and 73% of statistical analysis. Conclusion There is growing interest in adulthood outcomes of ChildSARDs, as seen in the increasing number of publications. However, the majority of studies demonstrated moderate to high risk-of-bias. Most studies used cross-sectional study designs, which limits robust assessment of prognostic factors. Readers should be aware of design limitations of interpreting results from current studies. 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.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.100 | 0.337 |
| Meta-epidemiology (narrow) | 0.002 | 0.002 |
| Meta-epidemiology (broad) | 0.013 | 0.011 |
| Bibliometrics | 0.046 | 0.036 |
| Science and technology studies | 0.002 | 0.003 |
| Scholarly communication | 0.009 | 0.007 |
| Open science | 0.003 | 0.005 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.006 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".