关键词: 全身型幼年特发性关节炎, 巨噬细胞活化, 发病机制, 固有免疫

Abstract: Juvenile idiopathic arthritis（JIA） is a chronic systemic disease in children with disease onset prior to age 16 and is featured by chronic arthritis，accompanied by other tissue and organ damage，with exclusion of arthritis caused by other diseases. Compared with other types， systemic JIA（sJIA） is characterized by unique clinical features such as remittent fever， skin rash，serous effusions， hepatomegaly， splenomegaly， lymphadenopathy and multiple organ damage. Some children are also at risk of life-threating complications including macrophage activation syndrome（MAS）. In addition，sJIA is also featured by its laboratory parameters，for instance， significantly elevated levels of inflammatory markers［C reactive protein（CRP），erythrocyte sedimentation rate（ESR）］， significantly elevated levels of peripheral blood white cells and neutrophils as well as absence of autoantibodies. Therefore， it is generally believed that the pathogenesis of sJIA is different from other subsets and sJIA represents a distinct category of disease. Nevertheless，the pathogenesis of sJIA still remains elusive. It is a unique category of arthritis， similar to autoinflammatory diseases， which is probably due to disturbed innate immune system induced by certain infection in genetically susceptible individuals.

Key words: systemic juvenile idiopathic arthritis（sJIA）, macrophage activation syndrome（MAS）, pathogenesis, innate immune system