摘要
成人Still病(AOSD)是一种少见多因素自身炎症性疾病,以发热、皮疹、关节炎/痛、淋巴结肿大、白细胞升高等为主要表现。AOSD还可出现严重并发症如巨噬细胞活化综合征,危及患者生命。最近一年,随着对AOSD发病机制的深入研究以及生物制剂和小分子靶向药物的广泛应用,人们对AOSD有了更多新的认识。本文将对近一年来在AOSD发病机制、临床表现和治疗等方面的进展进行述评,并对未来研究方向提出希望。
Adult onset Still′s disease(AOSD)is a rare polygenic autoinflammatory disease mainly manifesting as high-spiking fever,rash,arthritis/arthralgia,lymphadenopathy,and leukocytosis.More importantly,life threatening macrophage activation syndrome may occur in AOSD patients.Recently,with the development of research in pathogenesis and therapy strategies of biological agents and small molecule targeted drugs,we have new recognition of AOSD.In this commentary,we attempt to place this syndrome in perspective,including data in the past year on pathogenesis,clinical and laboratory features and therapy.
作者
沈敏
曾小峰
Shen Min;Zeng Xiaofeng(Department of Rheumatology and Clinical Immunology,Chinese Academy of Medical Sciences&Peking Union Medical College National Clinical Research Center for Dermatologic and Immunologic Diseases,Ministry of Science&Technology State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,Beijing 100730,China)
出处
《中华医学杂志》
CAS
CSCD
北大核心
2021年第25期1949-1952,共4页
National Medical Journal of China
基金
国家重点研发计划精准医学专项(2017YFC0907601,2017YFC0907602,2017YFC0907603,2017YFC0907604,2017YFC0907605)
中国医学科学院医学与健康科技创新工程项目(2019-I2M-2-008)
中国医学科学院基本科研业务费(2019PT330004)
北京市科学技术委员会资助项目(Z201100005520022-23,25-27)。
