Abstract

Systemic juvenile idiopathic arthritis (sJIA) is a severe orphan autoimmune disease characterized by fever, polymorphic skin rash, hepatosplenomegaly, serositis, generalized lymphadenopathy, and destructive arthritis. Proinflammatory cytokines, including interleukin-1 (IL-1), IL-6, IL-17, IL-18, and tumor necrosis factor (TNF), play a key role in sJIA pathogenesis. sJIA is refractory to conventional immunosuppressive antirheumatic drugs. Before the development of biologicals, sJIA patients had to receive corticosteroids constantly. The implementation of biologicals, such as tocilizumab (IL-6 inhibitor) and canakinumab (IL-1 inhibitor) into clinical practice radically changed the disease course, reduced the need for corticosteroids, and significantly decreased the risk of disability. The administration of biologicals at sJIA onset ensured faster achievement of inactive disease or remission compared to conventional therapy, as well as more patients responding to them. Implementation of biologicals into clinical practice dictated the need to identify optimal conditions for initiating therapy in sJIA patients to improve treatment efficacy and safety. Key words: biologicals, canakinumab, predictors, systemic juvenile idiopathic arthritis, tocilizumab, cytokines

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