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

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. Key words: Adult-onset Still's disease;  Pathogenesis;  Diagnosis;  Treatment