文献简介

出版社:

作  者:

编  号:

关键字:系统性硬化症;流行病学;预后;风险因素

年  份:2019   点击量:34

文献摘要 全文翻译

Systemic sclerosis (SSc) is a chronic immune-mediated connective tissue disease with heterogeneous organ involvement. New classification criteria were developed allowing disease identification even before the onset of its hallmark, skin fibrosis. Incidence and prevalence vary among reports and are influenced by methodology. Despite earlier diagnosis, mortality of SSc is still considerable, mainly because of cardiopulmonary causes. Genetic predisposition is entangled and implies genes of the major histocompatibility complex and also other loci related to immune regulation. Known environmental risk factor is exposure to organic solvents and silica, but no single risk factor has emerged. Disease outcome measures including patient-reported outcomes have been proposed and validated, and their use is expected to contribute to measure treatment response in clinical trials. Because of the low frequencies and the high heterogeneity of the disease, large multicenter research collaborations are envisaged to achieve advancement in SSc management.

系统性硬化症(SSc)是一种慢性免疫介导性结缔组织疾病,累及不同器官。制定了新的分类标准,甚至在其标志-皮肤纤维化-出现之前就可以对疾病进行识别。遗传易感性复杂,意指主要组织相容性复合体基因以及与免疫调节相关的其它基因位点。发病率和患病率因报告而异,并受方法学影响。尽管可早期诊断,但SSc的死亡率仍然相当高,主要是因为心肺原因。已知的环境风险因素是接触有机溶剂和二氧化硅,但未出现单一风险因素。已提出并验证了疾病预后测量方法(包括已报道的患者预后),其使用有助于在临床试验中测量治疗反应。由于该疾病发病率低且高度异质性,因此需建立大型多中心研究以在SSC管理方面取得进展。