Department of Medicine, Division of Connective Tissue Diseases, University of Tennessee Health Science Center, Room G326, Memphis, TN 38163, USA.
Expert Opin Pharmacother. 2010 Apr;11(5):789-806. doi: 10.1517/14656561003592177.
Systemic sclerosis (SSc) is an uncommon autoimmune disease with variable degrees of fibroproliferation in blood vessels and certain organs of the body. There is currently no cure. The purpose of this article is to review the current literature regarding pathogenesis and treatment of complications of SSc.
All available articles regarding research related to SSc pathogenesis and treatment listed in the PubMed database were searched; relevant articles were then reviewed and used as sources of information for this review.
This review attempts to highlight for the reader some current thought regarding mechanisms of SSc pathogenesis and how autoimmunity relates to vascular changes and fibrogenesis of the disease, as well as providing a review of results of completed clinical trials and current ongoing clinical trials that address organ-specific or global therapies for this disease. This can aid physicians who provide medical care for patients with SSc.
SSc is a complex autoimmune disease, the pathogenesis of which, although not completely understood, is under active study; new insights into pathogenesis are continually being discovered. Although there is no effective disease-modifying treatment for patients with SSc, quality of life, morbidity and mortality can be improved by using targeted therapy directed at affecting the consequences of damage to lungs, blood vessels, kidneys and the gastrointestinal tract. Innovative approaches to treating SSc are under intense investigation.
系统性硬化症（SSc）是一种罕见的自身免疫性疾病，其血管和身体某些器官存在不同程度的纤维增生。目前尚无治愈方法。本文旨在回顾 SSc 并发症的发病机制和治疗的现有文献。
在 PubMed 数据库中搜索了与 SSc 发病机制和治疗相关的所有可用研究文章；然后对相关文章进行了回顾，并将其作为本综述的信息来源。
这篇综述试图向读者强调一些目前关于 SSc 发病机制的机制的思考，以及自身免疫与疾病的血管变化和纤维化之间的关系，并对已完成的临床试验结果和目前正在进行的临床试验进行综述，这些试验涉及针对这种疾病的特定器官或全身性治疗。这可以帮助为 SSc 患者提供医疗服务的医生。
SSc 是一种复杂的自身免疫性疾病，其发病机制虽然尚未完全了解，但正在积极研究中；对发病机制的新见解不断被发现。尽管对于 SSc 患者没有有效的疾病修饰治疗方法，但通过针对肺部、血管、肾脏和胃肠道损伤后果的靶向治疗，可以改善生活质量、发病率和死亡率。治疗 SSc 的创新方法正在深入研究中。
