Current and emerging biological therapy in adult-onset Still's disease.

Adult-onset Still's disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia, and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, or conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs). In this review, we focused on the roles of these cytokines in the pathogenesis of AOSD and summarized the current and emerging biological therapy.