Canakinumab for systemic-onset juvenile idiopathic arthritis/Still's disease-effectiveness and safety data from the BiKeR registry.

Objectives: In Germany, canakinumab is approved for the treatment of refractory systemic juvenile idiopathic arthritis (sJIA)/Still's disease. This study assesses the experience of sJIA/Still's disease patients treated with canakinumab in clinical practice. Methods: Data of 79 sJIA/Still's disease patients extracted from the BiKeR registry included patients' and disease characteristics. Three subgroups with disease durations before canakinumab initiation of ≤3 months, >3 to ≤12 months, and >12 months were compared, and 2 with canakinumab as first- and second- or more-line treatment. Results: Disease duration was ≤3 months before canakinumab initiation for 26 patients, >3 to ≤12 months for 24 patients, and >12 months for 29 patients. Thirty-nine patients received canakinumab as first-line treatment and 40 as second- or more-line treatment. Disease severity as assessed by the systemic Juvenile Arthritis Disease Activity Score-10 (sJADAS10) was highest for patients with a disease duration <3 months before canakinumab initiation. In the total cohort, after 3, 6, 12, 18, and 24 months, 86%, 81%, 81%, 75%, and 87% of patients reached inactive disease, respectively. Numerically, more patients reached the treatment goal of inactive disease when the disease duration was shorter before initiation of canakinumab. Minimal disease activity (sJADAS10 ≤6.0) did not differ among subgroups. Compared with second-line, more patients with first-line treatment reached inactive disease at months 6, 12, and 24 after canakinumab initiation. sJADAS10 at 12 months correlated significantly with disease duration before treatment. Conclusions: Canakinumab treatment led to high improvement rates and achievement of inactive disease state, particularly if they are administered early. Data from clinical practice confirm clinical study data.