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 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] Three-quarters of patients with sJIA treated with TCZ had higher height velocities (6.6 cm/year) [78] | Intravenous route Weight < 30 kg: 12 mg/kg/dose Weight > 30 kg: 8 mg/kg/dose 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] 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] Median time to achieve remission was 33 days 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] : At five-year-mark, 96% of patients had inactive disease and 76% of patients had inactive disease and not receiving medication [5] | Subcutaneous route 2 mg/kg/day 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: At 15 days, 84% of CAN receivers reached ACR30 response At the open-label part, 1/3 attained inactive disease, and 73% reached ACR 50 response In the withdrawal phase, relative risk reduction of flares was 64% lower in the CAN group 33% of patients received CAN discontinued CS | Subcutaneous route 4 mg/kg/dose Max dose: 300 mg 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 | 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. Time to respond was shorter in the rilonacept-treated group The CS has been weaned off in most of patients who received rilonacept. | Subcutaneous Should be given weekly 4.4 mg/kg loading dose and then 2.2 mg/kg weekly doses |