Pathogenesis,disease course,and prognosis of adult-onset Still's disease:an update and review
10.1097/CM9.0000000000000538
- Author:
Wang MENG-YAN
1
;
Jia JIN-CHAO
;
Yang CHENG-DE
;
Hu QIONG-YI
Author Information
1. Department of Rheumatology and Immunology
- Keywords:
Adult-onset Still's disease;
Biomarkers;
Disease course;
Pathogenesis;
Treatment
- From:
Chinese Medical Journal
2019;132(23):2856-2864
- CountryChina
- Language:Chinese
-
Abstract:
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.
