成人Still病发病机制、诊断和治疗进展

Advances of pathogenesis, diagnosis and treatment in adult-onset Still＇s disease

成人Still病（AOSD）是一种病因未明，以长期发热、一过性皮疹、关节炎／关节痛等为主要表现的少见全身炎症性疾病。近年来对其发病机制的研究显示炎性细胞因子，尤其是白介素18（IL-18），在其发病过程中起了重要作用。并有一些少见临床表现的报道。过去一直是一个排除性的诊断，近年有人提出了新的临床诊断标准，对不明原因发热患者中AOSD的诊断具有很高的特异性、阳性预测值、阴性预测值及较高的敏感性。基于对本病发病机制的新认识，新的治疗如抗肿瘤坏死因子α、抗IL-1、抗IL-6、抗CD20B细胞、抗核转录因子κB都取得了较理想的效果，也带来了耐药、不良反应等新问题。

Adult onset Still＇ s disease （AOSD） is a rare systemic inflammatory disorder with unclear aetiology and pathogenesis. The fundamental clinical manifestations include long-term fever, evanescent rash and arthritis/arthralgia. The important role of proinflammatory cytokines, especially interleukin 18 （IL-18）, in the pathogenesis is discovered in recent years. And one clinical scale is designed to distinguish AOSD from unclear fever patients. It is proved to be highly specific with great positive and negative predictive values and higher sensitivity. New therapies are established based on the new recognition to the pathogenesis, such as anti-tumor necrosis factor a, anti-IL-1, anti IL-6, anti CD20 B cell and anti nuclear factor-κB. But new problems such as resistance and adverse effect are found.