Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis...Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis,biomarkers,disease course,prognosis,and treatment in AOSD.Data sources:We retrieved information from the PubMed database up to July 2019,using various search terms and relevant words,including AOSD and Still's disease.Study selection:We included data from peer-reviewed journals.Both basic and clinical studies were selected.Results:Pathogenesis of AOSD involves genetic background,infectious triggers,and immunopathogenesis,mainly the activation of macrophages and neutrophils followed by a cytokine storm.Diagnosis and prognosis evaluation of AOSD is still challenging;therefore,there is an urgent need to identify better biomarkers.Biologic agents,including interleukin(IL)-ip,IL-6,and tumor necrosis factor-a antagonists in the treatment of AOSD,have good prospect.Conclusion:This review highlights the advances in pathogenesis,potential biomarkers,disease course,and treatment in AOSD.展开更多
基金This work was supported by grants from the National Natural Science Foundation of China(Nos.81671589,81871272).
文摘Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis,biomarkers,disease course,prognosis,and treatment in AOSD.Data sources:We retrieved information from the PubMed database up to July 2019,using various search terms and relevant words,including AOSD and Still's disease.Study selection:We included data from peer-reviewed journals.Both basic and clinical studies were selected.Results:Pathogenesis of AOSD involves genetic background,infectious triggers,and immunopathogenesis,mainly the activation of macrophages and neutrophils followed by a cytokine storm.Diagnosis and prognosis evaluation of AOSD is still challenging;therefore,there is an urgent need to identify better biomarkers.Biologic agents,including interleukin(IL)-ip,IL-6,and tumor necrosis factor-a antagonists in the treatment of AOSD,have good prospect.Conclusion:This review highlights the advances in pathogenesis,potential biomarkers,disease course,and treatment in AOSD.
