全身型幼年特发性关节炎发病机制及治疗进展

Advances in the Pathogenesis and Treatment of Systemic Juvenile Idiopathic Arthritis

全身型幼年特发性关节炎(s JIA)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,s JIA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s JIA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s JIA取得了较好的疗效。

Systemic juvenile idiopathic arthritis(s JIA) 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 s JIA,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 s JIA 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 s JIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective.