Introduction: Systemic juvenile idiopathic arthritis (sJIA) is a polygenic autoinflammatory disease. The pathophysiology is still unclear, it is now well known that innate immune mechanisms play a central role with overproduction of inflammatory cytokines. The increasedknowledge on the role of these cytokines has provided a change in the natural history of the disease with the introduction of the targetedtreatments. Remarkable results has been observed with canakinumab, an anti-interleukin-1β monoclonal antibody, in two clinical trials but little information are available in real life.Objectives: To evaluate clinical inactive disease rate and safety of canakinumab in Italian patients with sJIA.Methods: We have collected retrospectively clinical and laboratory data of patients with sJIA treated with canakinumab in 9 ItalianPaediatric Rheumatology centers. Clinically inactive disease (CID) at 6 months was defined according to Wallace criteria. We analyzed theeffect of canakinumab on fever, rash, number of actives joints, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) andphysician’s global assessment of disease activity score. Clinical and laboratory data were obtained using a standard data collection form.Results: Forty seven patients (26 F) were included in the analyses. The median age (range) at the diagnosis and at the beginning of treatment with canakinumab was 7.6 (1-14.7) and 10.2 (1.7-22.2) years, respectively. Twenty seven patients (57.4%) had been previouslytreated with other biologic agents (18 with anakinra, 1 with tocilizumab, 6 with both and 2 with etanercept), withdrawn for inefficacyin 15/27 (55.5%). Thirty patients (63.8%) were receiving concomitant treatment with glucocorticoids at the median dose (range) of 0.69(0.02-2.75) mg/kg/die. Thirty nine out of 47 patients had > 6 months of follow-up. Among these 39 patients, 27 (69.2%) achieved CID at 6months and 5/27 (18.5%) were still on glucocorticoids. Of the 30 patients who received concomitant glucocorticoids at baseline, 24achieved 6 months of follow-up and 12 (50%) of these were able to withdraw glucocorticoids. Minor adverse events were reported in 5/30 (16.6%) patients:upper respiratory tract infections in 4 and transient injection site reaction in 1. No cases of macrophage activationsyndrome was reported.Conclusion: Our results provide initial real world evidence of the efficacy of treatment with canakinumab in patients with sJIA. In our study the percentage of patients who reached CID at 6 months is slightly higher (69.2%) than reported at the end (from 3 months to one year) of the 2 published randomized trials (60%) (1). No serious adverse events were recorded in our population.
|Number of pages||1|
|Publication status||Published - 2019|