Biologic therapies in systemic juvenile idiopathic arthritis

ABSTRACT

Introduction: 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.

KEYWORDS:

• Anti-interleukin 1
• anti-interleukin 6
• anakinra
• biologic drugs
• systemic juvenile idiopathic arthritis
• tocilizumab
• treatment

Article highlights

• With the introduction of biologic drugs, the control of inflammation has improved in systemic juvenile idiopathic arthritis (SJIA).

• Anti-interleukin-1 (anti-IL-1) or anti-IL-6 agents appear to be the most effective current biologic treatment in SJIA patients.

• Anti-IL-1 treatment seems to be more effective when the systemic features predominate in SJIA.

• Anti-tumor necrosis factor drugs are not as effective as anti-IL-1 or anti-IL-6 treatment in SJIA.

• Anakinra seems to be an effective biologic treatment in macrophage activation syndrome, which is a severe complication of SJIA.

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Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.