Systemic juvenile idiopathic arthritis (JIA), also known as Still’s disease, is the most severe category within the group of chronic childhood arthritis which are grouped under the umbrella term of JIA. Systemic JIA has been considered a therapeutic orphan until few years ago when the disease was treated primarily with corticosteroids with the known side effect especially on child growth. More recently the availability of new treatment modalities with biologic agents such as anti IL 6 (tocilizumab) and anti IL1 (anakinra, rilonacept and canakinumab) therapies have greatly advanced the possibilities for these children to be adequately treated.
Two trials have been recently completed one with tocilizumab and the other with canakinumab.
In the first trial, 112 children with systemic JIA with or without fever were randomized to receive tocilizumab (8 mg/kg every 2 weeks for children over 30 kg of body weight and 12 mg/kg every 2 weeks for children below 30 kg intravenously) or placebo for 3 months followed by an open label extension. After 3 months there was a clear discrimination between tocilizumab and placebo and at the end of year 1 80% of the patients reached at least 70% JIA ACR improvement with 52% able to discontinue corticosteroids. Adverse event were mainly infections and laboratory abnormalities such as neutropenia and aminotransferase elevation. A total of 6 deaths occurred, (3 during the conduct of the study and 3 after).
There were also two canakinumab trials administered at a dose of 4 mg/kg/monthly subcutaneously. The first was a single dose double blind placebo controlled trial for 84 patients with fever at enrollment showing that up to 61% of the patients reached at least a JIA ACR 70 response already at day 15. The second Phase III trial was a classic withdrawal study design in which, after 32 weeks of open-label treatment with canakinumab, 100/177 patients who had at least a JIA ACR 30 response and underwent corticosteroid tapering were randomly assigned to continued treatment with canakinumab or to placebo in a double blind fashion until flared occurred. In the withdrawal part the risk of flare was 26% of patients in the canakinumab group versus 75% in the placebo group with corticosteroids discontinuation in 42/128 patients (33%). There were 7 cases of MAS, and infections were more frequent with canakinumab than with placebo. A total of 4 deaths occurred (2 during the trial and 2 after canakinumab discontinuation).
This lecture will describe the most recent evidence for the treatment of systemic JIA.
Disclosure of Interest None Declared
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