Initiate treatment with inflammatory modulators early in adult-onset Still’s disease
摘要
Autoinflammatory, adult-onset Still’s disease (AOSD) is, if not the same disease, along the same spectrum as systemic juvenile idiopathic arthritis, presenting with arthralgia, fever and rash. Key therapies are corticosteroid-sparing interleukin (IL)-1 and IL-6 blockers. NSAIDs and short-term, high-dose corticosteroids are still recommended, with the latter mandatory in severe AOSD, and/or when complications such as macrophage activation syndrome (MAS) occur. Emerging therapies based on small trials in patients with refractory-AOSD include Janus kinase inhibitors and tadekinig alpha. Interferon-γ antagonist emapalumab is effective in patients with Still’s disease and MAS.