Background Targeted treatments are becoming the mainstay for many autoinflammatory diseases, including the systemic juvenile idiopathic arthritis (sJIA). Canakinumab (CAB), a fully human anti-interleukin-1β (IL-1β) antibody, has recently been shown to be effective in refractory sJIA patients. Experience on the safety and efficacy of this treatment is still limited and completely lacking for Greek children with sJIA.

Objectives To assess the efficacy and safety of CAB administration in Greek children with sJIA.

Methods The recorded demographic, clinical, lab data were retrieved from the recently established “National Registry for sJIA under CAB”. Response was assessed according to the adapted American College of Rheumatology (ACR) pediatric criteria 1, 3, 6 and 12mos post-treatment.

Results Six children (M:F 3:3) from different country regions, aged 4-16.4 years, all with a systemic disease course have been recorded in the Registry so far. Four pts were previous non responders to Methotrexate (MTX) +prednisolone (PDN) and 2 of them were additionally receivers of etanercept (1/4) or anakinra (1/4) prior to CAB. The remaining 2 pts were either non compliant to anakinra (1) or steroid dependent (1). Their disease duration prior to CAB treatment was 0.6- 16.2 yrs (median 7.66). All pts received the drug within the 1st to 7th mo after their flare (median 1mo), 5/6 have completed a follow-up period of 12mo to date while the remaining one, 8mo. CAB was administered (4mg/kg/mo, sc) together with PDN (6/6), plus MTX (5/6) or Leflunomide (1/6). At baseline, the systemic features were fever (6/6), rash (4/6), lymphadenopathy (2/6) and serositis (1/6). The range of active joints was 0-12 (median 2) and of the CRP 4-195mg/dL (median 71, cut off value ≤5). All pts responded to CAB without any adverse events; 5/6 achieved disease remission 1mo post-treatment, and the other one in the 3rd mo. Fever disappeared in 5/6 pts after the first drug administration and in the other one after the 3rd mo, while the rest systemic features, 1st mo post-treatment. The 6 pts achieved the following post-treatment ACRped 30/50/70/100 scores: 1st mo 2/0/1/3, 3rd mo 0/2/1/3, 6th mo 0/1/2/3 and 12th mo 0/1/1/3. 4/6 pts were able to discontinue PDN within the first to 6th mo, 1pt achieved a 20% PDN reduction and the latter one is still under PDN, but in clinical remission. No correlation was found between the level of ACRpedi and the disease duration prior to CAB administration for any time point of the study.

Conclusions CAB was well tolerated by all 6pts, had a steroid sparing effect and a rapid efficacy in the vast majority of them. This on-going registry will be enriched and will follow-up all CAB recipients in a long-term base.

1. Ruperto N, Quartier P, Wulffraat N et al. A phase II study to evaluate dosing and preliminary safety and efficacy of canakinumab in systemic juvenile idiopathic arthritis with active systemic features.Arthritis Rheum. 2011 Sep 27. doi: 10.1002/art.33342. [Epub ahead of print]

Disclosure of Interest M. Trachana Grant/Research support from: Abbott/Novartis/Pfizer, P. Pratsidou-Gertsi Grant/Research support from: Abbott/Novartis/Pfizer, O. Vougiouka: None Declared