Abstract

Systemic-onset juvenile idiopathic arthritis (SoJIA) represents up to 20% of chronic inflammatory arthritis cases in childhood. The clinical manifestations of this disease are unique compared with other forms of juvenile idiopathic arthritis, and many SoJIA patients do not respond to available therapies, including antitumor necrosis factor agents. It has recently been demonstrated that the serum of SoJIA patients contains an interleukin (IL)-1-inducing factor, and an excess of IL-1β is secreted upon activation of SoJIA leukocytes in vitro, suggesting that IL-1 might represent a potential therapeutic target in this disease. Indeed, administration of an IL-1 receptor antagonist induced clinical remission and corrected the laboratory abnormalities in a group of patients who had been refractory to conventional therapies, supporting the idea that IL-1 is an important mediator of the disease.

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