全身型幼年特发性关节炎发病机制研究进展

幼年特发性关节炎(JIA)是一组16岁以前起病,原因不明,以慢性关节炎为主要特征,可伴有其他组织、器官损害的慢性全身性疾病,并除外其他疾病所致关节炎.与其他类型JIA相比,全身型JIA(sJIA)常伴有特殊的临床表型,如弛张高热、皮疹、浆膜腔积液、肝脾淋巴结肿大及多器官损害,少数甚至伴发巨噬细胞活化综合征(MAS).此...

Update on the pathogenesis of systemic juvenile idiopathic arthritis

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 lifethreating 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.