Drug and studies included in this summary

Mode of action

Effectiveness as per studies included in this summary

Dosage and route

Tocilizumab (TCZ) [22] [78]

Human recombinant monoclonal

anti-IL-6 receptor

An RTC study recruited sJIA patients who had uncontrolled JIA despite NSAIDs and corticosteroids usage; and received TCZ versus a placebo

Ÿ By 3 months, 85% of sJIA patients who received TCZ reached ACR30 response and no fever, 71% reached ACR70 response and 37% reached ACR90 response compared to less than 25% who become afebrile and has ACR 30 in the placebo group [22]

Ÿ Three-quarters of patients with sJIA treated with TCZ had higher height velocities (6.6 cm/year) [78]

Ÿ Intravenous route

Ÿ Weight < 30 kg: 12 mg/kg/dose

Ÿ Weight > 30 kg: 8 mg/kg/dose

Ÿ Should be given every 2 weeks

Anakinra (ANK) [2] [5] [21]

Human recombinant anti-IL-1 receptor

1) RTC study was done on sJIA pateints who had persist ently active disease despite CS or NSAIDs [21]

Ÿ By one month, 92% of patients receiving ANK reached ACR30 response and no fever, 58% reached ACR50 response and 42% reached ACR 70 response [21]

Ÿ Median time to achieve remission was 33 days

Ÿ 76% of patients of ANK users had inactive disease at 1 year and 52% of ANK users had inactive disease while on medication [5]

2) A prospective cohort studied newly diagnosed sJIA pateints who treated with ANK as initial treatment [5] :

Ÿ At five-year-mark, 96% of patients had inactive disease and 76% of patients had inactive disease and not receiving medication [5]

Ÿ Subcutaneous route

Ÿ 2 mg/kg/day

Ÿ Range: 1-4 mg/kg/day

Canakinumab (CAN) [76] [77]

Monoclonal anti-IL-1 Beta

An RTC included sJIA patients with active disease, could be in low dose daily CS or NSAIDs, included 190 patients:

Ÿ At 15 days, 84% of CAN receivers reached ACR30 response

Ÿ At the open-label part, 1/3 attained inactive disease, and 73% reached ACR 50 response

Ÿ In the withdrawal phase, relative risk reduction of flares was 64% lower in the CAN group

Ÿ 33% of patients received CAN discontinued CS

Ÿ Subcutaneous route

Ÿ 4 mg/kg/dose Max dose: 300 mg

Ÿ Should be given every 4 weeks

Rilonacept [84] [85]

Did not receive JIA approval

Investigational

complex protein consisting of three fused parts

Acts against IL-1 alpha and IL-1 beta

Ÿ At 4 weeks, 60% and 40% of the rilonacept-treated group had ACR50 response and ACR70 response, versus 30% and 12% of those in the placebo group respectively.

Ÿ Time to respond was shorter in the rilonacept-treated group

Ÿ The CS has been weaned off in most of patients who received rilonacept.

Ÿ Subcutaneous

Ÿ Should be given weekly

Ÿ 4.4 mg/kg loading dose and then 2.2 mg/kg weekly doses