THU0575 Anakinra for first line steroid free treatment in systemic onset juvenile idiopathic arthritis

Abstract

Background: Systemic juvenile idiopathic arthritis (sJIA) is characterised by arthritis accompanied or preceded by systemic autoinflammation. High-dose steroids has been the mainstay of therapy with proven effectiveness but also with side-effects. In many patients a chronic course with destructive arthritis long-term cannot be prevented. Objective(s): In patients naive for steroids, a steroid-free treatment may allow reconstitution of an impaired NK-cell function and probably remission of sJIA. Method(s): First experience with first line Anakinra without steroids in 9 consecutive patients is reported. Result(s): All patients presented with ongoing spiking fever and rash and further features of sJIA, high CRP, S100 and IL18 (table 1). Daily sq. injections of Anakinra 2 mg/kg for 3 months resulted in complete remission in 4 and partial response in two children presenting with an oligoarticular involvement. One patient with typical sJIA and very high S100 (MRP8/9) levels did not respond to Anakinra nor to Canakinumab. Two patients presented with polyarthritis. One had no response, the other showed a minor response but improved on steroids and was later treated with tocilizumab. One of the oligoarticular patients with an initial partial response had a flare upon anakinra captured by increased dosing (4 mg/ kg) but finally developed macrophage activation syndrome. Anakinra was discontinued after 3 months in 3 of the 4 responders. Two remained in drug free remission while the remainder flared several months later and retreatment was instituted. The patients with polyarticluar involvement first received corticosteroids and were later both treated successfully with the IL-6 inhibitor. Conclusion(s): Experience with first line steroid free treatment with Anakinra for sJIA is presented. A complete remission was reached in 4 cases with oligoarticular involvement. In 3 further cases improvement was observed and 2 had no response including one who also failed Canakinumab. A toddler with a particular response to Anakinra later on developed MAS. One patient did not respond to both IL-1 inhibitors. Thus, steroid free treatment regimen with Anakinra is feasible and resulted into remission in most but not all patients. Aside, unwarranted effects of long lasting steroid application were avoided.