Autoinflammatory & rare systemic diseases
The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry
Authors' conclusion
The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.
Study context
- Population
- Patients with Still disease in the GIRRCS AOSD Study Group and the AIDA Network Still Disease Registry (mean ± SD age 36.6 ± 17.3 years; male 44.4%)
- Participants
- 597 patients
- Outcome
- Life-threatening evolution (mortality and/or macrophage activation syndrome) predicted by the systemic score
Methodological detailsmulticentre, observational
- Multicentre
- Observational
Main finding
As reported in the abstract
Life-threatening evolution
Systemic score as continuous variable, OR 95% CI 1.07-1.42, P = 0.004
1.24
Systemic score ≥7, OR 95% CI 1.81-6.25, P < 0.001
3.36
Observational data: associations, not effects.
Further findings
Life-threatening evolution by systemic score component
- Liver involvement, OR 95% CI 1.48-2.67, P = 0.031
- 1.68
- Lung disease, OR 95% CI 1.14-4.49, P = 0.042
- 2.12
Original publication
The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry. Arthritis Rheumatol. Published 15 May 2024. doi:10.
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