| Summary: | Juvenile idiopathic arthritis (JIA) is a relatively common, chronic childhood disorder, with clinical manifestations mainly related to joint inflammation and including joint effusion, joint line tenderness and warmth, restricted range of movement, and limitation of movement secondary to pain. Systemic onset JIA (sJIA) is a subtype of the disease accounting for approximately 4% to 15% of patients, and is defined as arthritis in one or more joints for at least 6 weeks in a child younger than 16 years with or preceded by fever of at least 2 weeks that is documented to be daily for at least 3 days and accompanied by one or more of the following: evanescent erythematous rash, generalized lymphadenopathy, hepatomegaly or splenomegaly, and serositis. Patients with sJIA experience an intense inflammatory state leading to a particularly refractory course and persistent disease. As a result, these patients are at high risk for serious complications such as joint damage and growth impairment, as well as macrophage activation syndrome (MAS), a life-threatening complication developing in 10% to 15% of children with sJIA and associated with a mortality rate that may reach 20%. Canakinumab is a fully human monoclonal antibody that selectively binds and neutralizes interleukin-1 beta, which plays a key role in the inflammatory process of sJIA. Canakinumab has a Health Canada indication for the management of active sJIA in patients 2 years and older. The manufacturer has requested that canakinumab be evaluated for reimbursement for the management of active sJIA in patients 2 years and older who are contraindicated to, or have discontinued, any biologic therapy for lack of efficacy or intolerance. The objective of this report was to perform a systematic review of the beneficial and harmful effects of canakinumab subcutaneous injection for the management of active sJIA in patients aged e 2 years
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