In a long-term extension of two phase III studies assessing the safety and efficacy of canakinumab (a fully human anti-IL-1β monoclonal antibody) in patients with active systemic juvenile idiopathic arthritis (JIA), treatment efficacy at 6 months was maintained for up to 5 years. No new safety findings were reported and treatment was associated with glucocorticoid discontinuation or substantial reduction in glucocorticoid dose. Overall, 102 patients (58%) discontinued canakinumab, mainly because of treatment inefficacy. A higher rate of discontinuation was noted for late responders than for early responders (81% versus 29%), indicating that early response is a predictive factor of long-term outcome.