Anakinra as a first-line therapy for systemic juvenile idiopathic arthritis when nonsteroidal anti-inflammatory drug treatment fails: A single-center French retrospective study.

Introduction: Anakinra has dramatically improved the management of systemic juvenile idiopathic arthritis (SJIA) over the last decade. Nevertheless, management remains inconsistent; corticosteroids are still frequently used. We analyzed the course of SJIA in children treated with anakinra according to the time of treatment initiation after disease onset.

Method: Children with SJIA treated with anakinra between 2006 and 2020 were included in this single-center, retrospective observational study.

Results: Twenty-four children received anakinra at a median time of 58 (range 12-2940) days after SJIA onset, all after failure of nonsteroidal anti-inflammatory drug (NSAID) treatment. Eighteen were males and the median age at disease onset was 6.04 (range 0.8-13) years. The median follow-up time was 3.5 (range 0.5-10.8) years after treatment initiation. At the last follow-up, remission attributable to anakinra was observed in 18/24 (75%) children and treatment-free remission was observed in 12 (67%). For each child, the response to anakinra was the same at 3 months and at the last follow-up. The 15 children treated with anakinra within the first 3 months after disease onset exhibited better remission (93%) than did the 9 children treated after 3 months (44%) (p = 0.015) and the former received fewer corticosteroids (7% versus 67%) (p = 0.004). One child with long-standing disease died of the disease.

Conclusions: Early anakinra initiation within the first 3 months of SJIA onset after NSAID failure ensures long-term remission and reduces corticosteroid use. Anakinra should not be continued for more than 3 months in nonresponding children.