Abstract

The American College of Rheumatology (ACR) updated the 2011 recommendations for juvenile idiopathic arthritis (JIA) in 2013 and focused on the treatment of systemic-onset juvenile idiopathic arthritis (SOJIA). According to the clinical feature of the SOJIA, the subtype is developed to 3 phenotypes: (1) phenotype of systemic JIA with active systemic features and varying degrees of synovitis; (2) phenotype of systemic JIA without active systemic features and with varying degrees of active synovitis; (3) phenotype of systemic JIA with features concerning for macrophage activation syndrome (MAS). The treatment recommendation was made according each phenotype, including initial therapeutic options and therapeutic options for continued disease activity.And uncertain or inappropriate options for continued disease were listed alphabetically.Development of treatment recommendations for children with SOJIA and features of MAS is particularly challenging.However, it is anticipated that in the near future, with research of the disease process, the better recommendation may be made. Key words: Juvenile idiopathic arthritis, systemic-onset; Macrophage activation syndrome; Treatment; Update

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