系统性硬化症发病机制的研究进展  

Research Progress in Pathogenesis of Systemic Sclerosis

作　　者：杨书琦 张慧芳 宋书林[2] 余旻[1] 

机构地区：[1]三峡大学人民医院宜昌市第一人民医院重症医学科,湖北宜昌443000 [2]三峡大学人民医院宜昌市第一人民医院风湿免疫科,湖北宜昌443000

出　　处：《医学综述》2018年第1期17-21,共5页Medical Recapitulate

基　　金：国家自然科学基金(81501045);湖北省自然科学基金(2014CFA073)

摘　　要：系统性硬化症(SSc)是一种以影响皮肤和内脏为主的结缔组织病,其特点为血管和免疫功能紊乱引起纤维化,临床上可以观察到皮肤局限或弥漫性增厚变硬,并进一步损伤多个器官。其中,免疫系统的紊乱贯穿该病的全过程,在疾病的发生、发展中起重要作用。SSc的发病机制暂不明确,所以目前尚未有针对SSc的有效治疗方案。研究者们进行了大量的临床及基础实验,试图从遗传、环境、免疫细胞、细胞因子、胶原及血管功能紊乱等多个角度进行研究,以为未来新的治疗提供可能。Systemic sclerosis （SSc）, is a connective tissue disorder that affects the skin and internal organs, character- ized by vascular and immune dysfunction, with partial or diffuse skin thickening and stiffness as clinical manifestations, which can further cause organic damage. The immune system disorder has played an important role throughout the develop- ment and progression of SSc. There is no effective therapy for SSc, because the pathogenesis and mechanism of SSc have been unknown. Researchers have carried out a large number of clinical trials and basic experiments, trying to study from the perspectives of genetics, environmental, immune cells, cytokines, collagen, vascular dysfunction and other aspects, to provide new possibilities for future treatment.

关 键 词：系统性硬化症 硬皮病 免疫系统 纤维化 

分 类 号：R593.25[医药卫生—内科学]