LAUSR

OBJECTIVES To report the efficacy and safety of canakinumab treatment in Japanese patients with systemic juvenile idiopathic arthritis over a 48-week study period. METHODS Patients were administered canakinumab 4 mg/kg (maximum dose 300 mg) every 4 weeks, with no dose adjustments. The key outcomes measures included adapted American College of Rheumatology paediatric 30/50/70/90/100 response, proportion of patients with inactive disease, and corticosteroid-tapering. RESULTS In total, 16/19 (84.2%) patients received canakinumab for ≥96 weeks reaching end-of-study visit without premature discontinuation. Regardless of the level of joint involvement at baseline, high adapted American College of Rheumatology paediatric responses were observed throughout the study; at end-of-study, adapted American College of Rheumatology paediatric 90/100 response rates were 84.2%/63.2%, respectively. The proportion of patients who successfully tapered corticosteroids at end-of-study was 66.7% (12/18), of which 10 patients were steroid-free. The most common adverse events were infections (238.3 events/100 patient-years). Serious adverse events were observed in 52.6%. One event adjudicated as possible macrophage activation syndrome was preceded by sJIA flare. No deaths were reported. CONCLUSIONS Canakinumab treatment resulted in a sustained treatment response in systemic juvenile idiopathic arthritis patients over 48 weeks and was associated with corticosteroid-tapering in the majority of patients. No new safety findings were reported.