Biologic therapies in systemic juvenile idiopathic arthritis

Abstract

ABSTRACTIntroduction: Systemic juvenile idiopathic arthritis (SJIA) is characterized by fever, rash, arthritis, lymphadenopathy, hepatosplenomegaly, and serositis. It is frequently considered an autoinflammatory syndrome. There is interplay between different pro-inflammatory cytokines in SJIA pathogenesis. Sufficient control of inflammation is crucial to prevent complications. Treatment strategies in SJIA have changed dramatically with the introduction of increasing number of biologic agents. These modern drugs have improved the inflammation control and reduced side effects of traditional long-term treatments.Areas Covered: This review focuses on the biologic treatment in SJIA patients. The studies on biologic drug use in SJIA, the safety issues, current recommendations, and treatment for macrophage activation syndrome (MAS) are summarized.Expert Opinion: Biologic drugs blocking interleukin-1 (IL-1) or IL-6 appear to be the most effective current biologic treatment in children with SJIA. Anti-IL-1 treatment seems especially effective in SJIA patients with prominent active systemic features. Tumor necrosis factor inhibiting drugs are not as effective as anti-IL-1 or anti-IL-6 agents in SJIA treatment. For MAS, which is a severe complication of SJIA, the combination therapy with high dose corticosteroids, cyclosporine, and anakinra is likely to become the treatment of choice.