Background/Purpose Systemic Juvenile Idiopathic Arthritis (sJIA) is classified as an acquired autoinflammatory disease. The interleukin-1 and interleukin-6 play a pivotal role in pathogenesis of this disease. The systemic manifestations as well as arthritis in sJIA are related to interleukin-6 action. Tocilizumab is effective drug for the treatment of systemic arthritis refractory to immunosuppressive drugs.
Objectives: To evaluate safety and efficacy of tocilizumab treatment in children with systemic juvenile idiopathic arthritis.

Methods 829 patients with sJIA were included in the register. 212 received tocilizumab. Analysis of efficacy and safety tocilizumab therapy was performed in 75 patients, whom follow up period was 2 years. Median age was 7,5 years (range; 0,9 to 15 years) and median disease duration was 2,5 years (range; 0.3 to 12 years). Tocilizumab was administrated intravenously at a dose of 8-12 mg/kg every 2 weeks during 2 months then every 4 weeks. All patients received DMARDs, 38 patients received prednisolone at dose 9.5 (6.5; 12) mg/day. Efficacy end points included the American College of Rheumatology (ACR) Pediatric criteria for improvement 30 (ACR30), ACR50, ACR70, ACR90 and criteria of inactive disease and remission. During the follow-up period, significant side-effects were sought and the reduction in oral prednisolone was recorded too.

Results The ACR Pedi 30, 50, 70 and 90 improvement were achieved by 100%, 75%, 58% and 30% of patients at Week 12 (n=73), by 100%, 90%, 80% and 55% of patients at Week 24 (n=72) and by 100%, 100%, 95% and 70% of patients at Week 52 (n=60), by 100%, 98%, 98% and 85% of patients at Week 104 (n=52), respectively. Inactive disease was achieved by 18/75 of patients at week 12, by 36/75 of patients at week 24, by 42/75 of patients at week 52, by 44/75 of patients at week 104. Remission was achieved by 44/75 of patients at Week 104. The mean dose of oral glucocorticoid was decreased from 0,5 (0,4; 0,7) to 0,1 (0,04; 0,2) mg/kg/day at week 52 (p<0.001), was dicontinued in 5 patients by Week 104 . The frequently observed non-severe adverse events were nasopharyngitis, upper respiratory tract infections and gastroenteritis. No cases of opportunistic infections, malignancies or death were reported. There were three cases of pneumonia and cellulitis. 30 patients had incidences of neutropenia, 2 – trombocytopenia, 15 – high level of ALT and AST. Tocilizumab treatment was discontinued in 23 patients during the follow-up 52 weeks period. The causes for cancellation were relapse of disease (n=9), lack of efficacy (n=8), self-cancellation by parents because of remission (n=2), parent's refusal (n=1), infusion reaction (n=2) and Crohn's disease (n=1). 7 patients were switched to canakinumab, 3- to rituximab and 4 – to antiTNF blockers. Survival of tocilizumab treatment was 96% at Week 24, 81% - at Week 52, 69% - at Week 104.

Conclusion

Tocilizumab induced remission of extra-articular manifestations, arthritis and normalized laboratory parameters of the disease activity without initiation of treatment with oral prednisolone and increase its dose, thus avoiding severe irreversible complications of glucocorticoid therapy. 

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/tocilizumab-therapy-in-children-with-systemic-juvenile-idiopathic-arthritis-data-from-russian-register-of-sjia/