Safety and efficacy of anakinra as first-line or second-line therapy for systemic onset juvenile idiopathic arthritis - data from the German BIKER registry.

: The IL-1 receptor-antagonist anakinra is recommended for the treatment of systemic juvenile idiopathic arthritis (sJIA) and was recently approved for first-line treatment. Long-term data from clinical practise are scarce. : SJIA patients from the German biologics in pediatric rheumatology (BIKER) registry starting anakinra were grouped into two cohorts: Patients in the first-line cohort received no prior sJIA treatment except NSAID and a maximum of 3 days of steroids. Second-line cohort patients were pre-treated with steroids; DMARDs or biologics. Patient characteristics, disease-activity parameters, efficacy, and safety-parameters were compared. : Until December 2018, 51 anakinra patients were documented, representing 117.96 patient-years. Mean disease duration was 3.5 (± 3.8) years. At baseline, all anakinra first-line users had active systemic disease compared to 82% in the second-line users. Significant JADAS-10 improvement at last follow-up was observed in both cohorts (p = 0.02, p = 0.0014). Substantial numbers of patients in both groups reached JADAS-MDA/JADAS-remission/inactive disease (66.7%50%50% in first-liners and 60%45%70% in second-liners). Rates of serious adverse events were comparable and consistent with the overall AE profile of anakinra in patients. : This analysis adds to the established safety profile of anakinra and demonstrates that anakinra is effective as first-line or second-line treatment.