The European Alliance of Associations for Rheumatology and Paediatric Rheumatology European Society now recognize systemic juvenile idiopathic arthritis and adult-onset Still’s disease as one condition—Still’s disease—unifying diagnosis and treatment strategies across age groups for the first time.

In May 2022, The European Alliance of Associations for Rheumatology (EULAR) and Paediatric Rheumatology European Society (PReS) jointly endorsed recommendations for diagnosing and managing Still’s disease, encompassing systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD). The international task force (TF) prioritized criteria for the disease spanning pediatric and adult patients, designating a singular term, “Still’s disease,” based on clinical and biological similarities between sJIA and AOSD.

The recommendations, published in Annals of the Rheumatic Diseases, center on four areas: disease designation, diagnostics, therapeutic targets, and complications like macrophage activation syndrome (MAS). Methods included systematic literature reviews to inform 14 guidelines, highlighting key principles. The TF emphasized “clinically inactive disease” (CID) and remission—defined as CID sustained for 6 months—as therapeutic goals, with initial treatment focusing on early interleukin-1 or -6 inhibitors and short-term glucocorticoids. MAS treatment necessitates high-dose glucocorticoids, IL-1 inhibitors, ciclosporin, and interferon-γ inhibitors. Considering severe lung disease cases, T-cell-directed immunosuppressants are suggested.

The TF highlighted the role of tertiary centers in managing complex cases, including those with MAS or lung disease. Additionally, they recommended intermediate targets at 7 days, 4 weeks, 3 months, and 6 months post-treatment initiation to aid in patient management.

Results from real-world experiences and limited randomized controlled trials support early IL-1/IL-6 inhibitor initiation, achieving CID without prolonged glucocorticoid use. The guidelines also incorporate quality indicators, with metrics focusing on remission rates at 6 months, early biological agent use, and referral to specialized centers for complex cases. 

Full disclosures can be found in the published recommendation.