成人Still病发病机制及治疗新进展

Progress on pathogenesis and treatment of adult-onset Still disease

成人Still病（AOSD）是一种病因未明的多因素自身炎症性疾病，临床表现复杂，以发热、皮疹、关节炎，痛、淋巴结肿大、白细胞升高等为主要表现。AOSD发病机制尚不明确，目前认为多种炎症细胞及因子参与其发病。AOSD的治疗以激素及免疫抑制剂为主。近年来，随着自身炎症性疾病的研究进展，以及生物制剂的研发上市，人们对AOSD的发病机制和治疗策略有了新的认识。本文针对AOSD的发病机制及治疗新进展予以综述。

Adult-onset Still disease （AOSD） is a polygenic autoinflammatory disease with uncertain etiology. The clinical manifestations are complex, including high-spiking fever, rash, arthritis, arthralgia, lymphadenopathy, and leukocytosis. The pathogenesis of AOSD is still unclear, whereas it has been confirmed that many inflammatory cells and eytokines take part in the development of AOSD. The treatment of AOSD is mainly based on corticosteroids and immunosuppressive agents. Recently, with the research progress of autoinflammatory diseases and the emerging biological agents, we have new knowledge on the pathogenesis and therapeutic strategies of AOSD. This article reviews the current progress in pathogenesis and therapy of AOSD.