摘要全身型幼年特发性关节(sJIA)是幼年特发性关节炎(JIA)的一个亚型,不同于JIA的其他亚型,sJIA的临床表现除关节炎外,全身表现特别突出,如弛张热、皮疹、肝脾大、浆膜炎等,与巨噬细胞活化综合征强相关.sJIA发病机制上主要与固有免疫异常有关,细胞因子白细胞介素(IL)-1、IL-6、IL-18及中性粒细胞、单核细胞、巨噬细胞等起重要作用.目前认为sJIA不是自身免疫性疾病,而是一个自身炎症性疾病.对sJIA发病机制的新认识带来治疗方法的进步,近年来,IL-1和IL-6拮抗剂靶向治疗取得了良好效果,为sJIA患儿远期预后带来了新希望.

abstractsSystemic juvenile idiopathic arthritis (sJIA) is systemic inflammatory disease classified as a subtype of juvenile idiopathic arthritis (JIA).Besides arthritis,it is characterised by systemic features such as spiking fever,skin rash,hepatosplenomegaly or serositis.It is becoming clear now that abnormalities in the innate immunity [cytokines such as interleukin (IL)-1,IL-6 and IL-18,and neutrophils and monocytes/macrophages rather than lymphocytes] play a major role in the pathogenesis of sJIA,distinguishing it from other JIA subtypes.Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS).Based on this,consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease.As a consequence of the progression in understanding the underlying mechanisms of sJIA,major changes in the management are evolving.Recently,remarkable improvement has been observed with IL-1 and IL-6 targeted therapies.These therapies might also change the long-term outcome of this disease.