Autoinflammatory & rare systemic diseases
Derivation and validation of four patient clusters in Still’s disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry
Authors' conclusion
Four patient clusters in Still's disease may be recognised by a multidimensional characterisation ('Juvenile/Transitional', 'Uncomplicated', 'Hyperferritinemic' and 'Catastrophic').
Study context
- Population
- Patients with Still's disease in GIRRCS AOSD-study group and AIDA Network Still Disease Registry
- Participants
- 349 patients
- Exposure
- Cluster 1 vs Cluster 2 vs Cluster 3 vs Cluster 4
- Cluster 1n = 115
- Cluster 2n = 128
- Cluster 3n = 31
- Cluster 4n = 75
- Outcome
- Derivation of patient clusters (age, systemic score, ESR, CRP, ferritin)
Methodological detailsK-means clustering
- K-means clustering
Main finding
As reported in the abstract
Patient clusters
- Four clusters; all mainly presented fever, skin rash and joint involvement
- Cluster 1: skin rash, myalgia, sore throat and splenomegaly
- Cluster 2: multiorgan manifestations less frequent
- Cluster 3: fever and joint involvement
- Cluster 4: myalgia, sore throat, liver involvement and life-threatening complications, leading to a high mortality rate
Observational data: associations, not effects.
Original publication
Derivation and validation of four patient clusters in Still’s disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry. RMD Open. Published 20 November 2023. doi:10.
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