CANAKINUMAB IN SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: REAL-LIFE DATA FROM A RETROSPECTIVE ITALIAN COHORT.

Objective: To evaluate in real-life the effectiveness and safety of canakinumab in Italian patients with systemic juvenile idiopathic arthritis (sJIA). Methods: A retrospective multicentre study of children with sJIA was performed. Clinical features, laboratory parameters and adverse events were collected at baseline, after 6 and 12 months from starting canakinumab. The effectiveness primary outcome was clinical inactive disease (CID) off glucocorticoids (GCs) treatment at 6 months. Results: A total of 80 children were analyzed from 15 Italian centers. Of the 12 patients who started canakinumab in CID while receiving anakinra, all maintained CID. Of the 68 with active disease at baseline, 57.4% achieved CID off GCs at 6 months and 63.8% at 12 months. In univariate analysis, the variables significantly related with non-response were number of active joints (NAJ) ≥5, history of macrophage activation syndrome (MAS) and disease duration. Multivariate analysis confirmed the association with non-response of NAJ ≥5 (OR 6.37 (95%CI 1.69-24.02), p= 0.006) and history of MAS (OR 3.53 (95%CI 1.06-11.70), p= 0.039). No serious adverse events were recorded in this series. There were two cases of MAS during canakinumab, leading to a rate of 2.9 episodes per 100 patient year. Conclusion: We confirm, in real-life, the efficacy of canakinumab in sJIA in a multicentric cohort. History of MAS and higher NAJ were associated with lower probability of achieving clinical inactive disease