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

Lancet RheumatolPublished 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
GroupAUROC
Development0.93
Preliminary validation0.85
External validation0.88

AUROC

Development0.93
Preliminary validation0.85
External validation0.88

Observational data: associations, not effects.

Further findings

OutcomePreliminary validation
Low disease activity (no fever and ≤1 of the other criteria) · 3 months and 6 months
AUROC at 3 months0.86
AUROC at 6 months0.94

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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