Recommendations for the therapeutic management of systemic juvenile idiopathic arthritis. Opinion of the Section of Developmental Age Rheumatology of the Polish Society of Rheumatology

Abstract

Advances in paediatric rheumatology determine the need to update management rules and implement the latest treatment standards for patients under our care. The following case provides the current therapeutic management of patients with systemic juvenile idiopathic arthritis (SJIA), taking into consideration the Treat to Target (T2T) concept, i.e. therapy aimed at achieving remission or, when this is not possible, an alternative goal is low disease activity. Simultaneously, the authors refer to the possibility of implementing these recommendations in Polish conditions, taking into consideration the B.33 drug programme, and they highlight that therapeutic options in SJIA should include both the individual course of the disease and available treatment options in the current legal and organisational system in Poland. In the early stages of the disease, nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticosteroids (GCs) are used for managing systemic symptoms of the disease. Advances in knowledge regarding the pathogenesis of SJIA have meant that current therapeutic strategies for the treatment of SJIA are based on targeted approaches using anti-cytokine
therapy that inhibits the activity of pro-inflammatory cytokines, primarily IL-1 and IL-6, and thus it is necessary to include IL-1 or IL-6 inhibitors in treatment when NSAIDs and GCs are not effective. It is considered necessary to include IL-1 inhibitors in the B.33 programme, primarily because of its causal effect. IL-1 is a mediator of the innate immune system and is a potent inducer of fever and inflammation. IL-1 inhibitors (iIL-1) enable GC-sparing effects with a reduction in their side effects. The overall response rate to IL-1 blockers (anakinra and canakinumab) is high, up to 90%, especially in patients with treatment-resistant forms of SJIA.