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Background:Still’s disease is a systemic auto-inflammatory disease with a pediatric form, sJIA, and an analogous condition in adults, adult-onset Still’s disease (AOSD). The role of interleukin-1 (IL-1) in the pathophysiology of Still’s disease is well established. Canakinumab, a monoclonal antibody against IL-1ß, is approved to treat patients with Still’s disease in Europe (sJIA and AOSD) and the United States (sJIA).Objectives:To study the efficacy of canakinumab in sJIA patients categorized by age, we performed an intention-to-treat analysis of pooled data from 5 clinical trials, as an addition to a previously communicated analysis including 3 of the studies1Methods:The age categories were children (2-Results:302 children, 82 young adolescents and 34 older adolescents and young adults were included in the analysis, with a mean disease duration of 922, 1708 and 2615 days, respectively. Prior therapy with other biologics was common, with anakinra used in 33%, 35% and 47% of patients in each group. Disease severity was comparable among groups, with the mean number of active joints ranging from 11.8 to 13.7. Adapted pediatric ACR responses revealed a rapid response to canakinumab, with all groups showing similar rates of responders at most time points (Table 1). In each age group, the proportion of patients with inactive disease progressively increased to Day 57. At all time points after Day 15, the 16-Table 1.Percentages of patients with Adapted pediatric ACR responses and inactive disease status over time*Time of treatment(Days)2 - a12 - b16-cACR301572.781.783.9 (%)2977.584.182.45776.282.988.28565.574.583.3ACR701551.558.364.5 (%)2961.962.270.65765.258.579.48558.661.875.0ACR1001521.625.012.9 (%)2929.530.535.35736.134.138.28534.130.933.3Inactive disease1519.030.019.4 (%)2934.134.147.15739.436.655.98536.743.452.2*Some studies did not include visits at Day 15 and/or 85. For Day 15, 29, 57 and 85 the respective denominators for each age group were:aN = 231, 302, 302, 232;bN = 60, 82, 82, 55;cN = 31, 34, 34, 24.Conclusion:The efficacy and safety profile of canakinumab was consistent in children, adolescents and young adults with sJIA. Since sJIA and AOSD represent pediatric- and adult- onset variants of the Still’s disease continuum, these results further support the therapeutic effect of canakinumab 4 mg/kg every 4 weeks in both children and adults with Still’s disease.References:[1]Feist et al.Clin Exp Rheumatol.2018;36(4):668-75.Disclosure of Interests:Pierre Quartier Consultant of: AbbVie, Chugai-Roche, Lilly, Novartis, Sanofi, Sobi, Speakers bureau: AbbVie, BMS, Chugai-Roche, Novartis, Pfizer, Sobi, Eugen Feist Consultant of: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi, Speakers bureau: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi, Daniel J Lovell Consultant of: Abbott (consulting and PI), AbbVie (PI), Amgen (consultant and DSMC Chairperson), AstraZeneca, Boehringer Ingelheim, Bristol-Myers Squibb (PI), Celgene, Forest Research (DSMB Chairman), GlaxoSmithKline, Hoffman-La Roche, Janssen (co-PI), Novartis (consultant and PI), Pfizer (consultant and PI), Roche (PI), Takeda, UBC (consultant and PI), Wyeth, Employee of: Cincinnati Children’s Hospital Medical Center, Speakers bureau: Wyeth, Hiroaki Umebayashi: None declared, Nicolino Ruperto Grant/research support from: Bristol-Myers Squibb, Eli Lily, F Hoffmann-La Roche, GlaxoSmithKline, Janssen, Novartis, Pfizer, Sobi (paid to institution), Consultant of: Ablynx, AbbVie, AstraZeneca-Medimmune, Biogen, Boehringer Ingelheim, Bristol-Myers Squibb, Eli Lily, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, Sanofi, Servier, Sinergie, Sobi, Takeda, Speakers bureau: Ablynx, AbbVie, AstraZeneca-Medimmune, Biogen, Boehringer Ingelheim, Bristol-Myers Squibb, Eli Lily, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, Sanofi, Servier, Sinergie, Sobi, Takeda, Hermine Brunner Consultant of: Hoffman-La Roche, Novartis, Pfizer, Sanofi Aventis, Merck Serono, AbbVie, Amgen, Alter, AstraZeneca, Baxalta Biosimilars, Biogen Idec, Boehringer, Bristol-Myers Squibb, Celgene, EMD Serono, Janssen, MedImmune, Novartis, Pfizer, and UCB Biosciences, Speakers bureau: GSK, Roche, and Novartis, Cornelia Dunger-Baldauf Employee of: Novartis, Stephanie Noviello Employee of: Novartis, sarah whelan Employee of: Novartis

Systemic-onset juvenile idiopathic arthritis (sJIA) is a chronic childhood disease associated with many complications.1–5 Treatments comprise non-steroidal anti-inflammatory drugs, corticosteroids, disease-modifying antirheumatic drugs and biologics,6–9 including tocilizumab, an interleukin-6 receptor monoclonal antibody.10 Results of a randomised, placebo-controlled, phase III trial of tocilizumab in sJIA patients at eight Japanese hospitals, as well as the first 48 weeks of an open-label extension, have been published.3 Protocols and amendments for this extension phase were approved by the Japanese Ministry of Health, Labor and Welfare and the institutional review board at each centre. Of 56 patients initially enrolled, six withdrew during the open-label, lead-in phase (anti-tocilizumab antibodies, three; anaphylactoid reaction, one; gastrointestinal haemorrhage, one; non-efficacy, one). During the double-blind phase, one patient from each treatment arm withdrew because of adverse events (AEs) but re-entered the open-label extension after the resolution of AEs. In total, 50 patients responding to tocilizumab and needing further treatment entered the open-label extension; two patients subsequently withdrew within the first year because of AEs. Herein, the long-term efficacy and safety of treatment through 144 weeks are presented. …