Figure 2.

Suggested treatment algorithm for patients with AOSD. Severe disease is defined as hospital admission needed, with organ involvement or life-threatening disease. Systemic symptoms: fever, weight loss, sore throat, skin rash, lymphadenopathy, hepatosplenomegaly. In case of predominant arthritis, IL-6 blockade may be preferred over IL-1 blockade. Low-dose glucocorticoid is ≤15 mg prednisone or equivalent. Double arrows indicate equivalent options and a decision should be made upon the physician’s discretion. GC refers to high dose glucocorticoid, i.e. prednisone 0.5–1.0 mg/kg or equivalent. ‘IL-1’ indicates IL-1 blockade; ‘IL-6’ indicates IL-6 blockade. MTX dosing according to EULAR; IL-1 blockade 100–300 mg subcutaneously or off label intravenously. AOSD: Adult onset Still’s Disease; GC: glucocorticoid; TNFi: TNF inhibitors