Pathogenesis, disease course, and prognosis of adult-onset Still's disease: an update and review.

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)-1β, IL-6, and tumor necrosis factor-α 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.