EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease

Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are one disease, to be designated by one name, Still's disease.

Two therapeutic targets: clinically inactive disease (CID) and remission, i.e. CID maintained for at least 6 months.

Optimal therapeutic strategy: early use of IL-1 or IL-6 inhibitors associated to short-duration glucocorticoid (GC).

Macrophage activation syndrome (MAS) treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors.

For severe lung disease in children with Still's disease, T cell directed immunosuppressants are suggested.

Key role of expert centres for difficult-to-treat patients.

All overarching principles and recommendations were agreed by over 80% of the task force (TF) experts with a high level of agreement.