Autoinflammatory & rare systemic diseases
Development and validation of clinical criteria for the definition of disease activity in adult-onset Still's disease: a cohort study
Authors' conclusion
Disease activity criteria in adult-onset Still's disease were developed to support the attainment of clinically inactive disease as the main treatment target in patient management.
Study context
- Population
- Consecutive patients aged ≥18 years fulfilling the Yamaguchi criteria for AOSD, Italian rheumatological centres (GIRRCS), Jan 1, 2022, to Dec 31, 2023187 GIRRCS + 41 CONSIDER
- Participants
- 130 / 57 / 41
- Exposure
- Development vs Preliminary validation vs External validation
- Developmentn = 130
- Preliminary validationn = 57
- External validationn = 41
- Outcome
- Performance of the developed disease activity criteria (AUROC)
Methodological detailsdevelopment and validation of clinical criteria, multicentre, external validation (CONSIDER trial baseline data), principal component analysis, longitudinal linear mixed model, AUROC, People with lived experience of AOSD involved in study design and implementation
- Development and validation of clinical criteria
- Multicentre
- External validation (CONSIDER trial baseline data)
- Principal component analysis
- Longitudinal linear mixed model
- AUROC
- People with lived experience of AOSD involved in study design and implementation
Main finding
As reported in the abstract
Active AOSD: fever and one of skin rash, arthritis, PGA ≥2 cm, CRP >10 mg/L; or no fever but ≥3 of the other criteria
| Group | AUROC |
|---|---|
| Development | 0.93 |
| Preliminary validation | 0.85 |
| External validation | 0.88 |
AUROC
Development0.93
Preliminary validation0.85
External validation0.88
Observational data: associations, not effects.
Further findings
| Outcome | Preliminary validation |
|---|---|
| Low disease activity (no fever and ≤1 of the other criteria) · 3 months and 6 months | |
| AUROC at 3 months | 0.86 |
| AUROC at 6 months | 0.94 |
Original publication
Development and validation of clinical criteria for the definition of disease activity in adult-onset Still's disease: a cohort study. Lancet Rheumatol. Published 25 March 2026. doi:10. 1016/ s2665-9913(25)00377-7
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