全身型幼年特发性关节炎发病机制及治疗进展

摘要

Systemic juvenile idiopathic arthritis ( sJIA ) is a special type of JIA. It is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. It is the most acute and severe type, which has a disproportionately high morbidity compared with other subtypes. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. Dysregulation of the innate immune system in sJIA results in increased production of inflammatory cytokines, leading to the distinctive clinical features of the disease. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective.%全身型幼年特发性关节炎( sJIA)是幼年特发性关节炎( JIA)的一种特殊类型,临床以发热、淋巴结肿大、关节炎、皮疹和浆膜炎为特征,与其他类型相比是最急性和最严重的一种类型,病死率高。近期研究表明,sJIA的系统炎症与固有免疫系统功能失调有关,属于自身炎症性疾病,固有免疫系统的失调致炎症细胞因子的增加而产生相应的临床症状。白细胞介素( IL)-1和IL-6在sJIA的发病机制中发挥了主要作用,而且用IL-1和IL-6拮抗药治疗sJIA取得了较好的疗效。