Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy.
Department of Clinical Medicine, La Sapienza University, Rome, Italy.
J Cell Mol Med. 2018 Jul;22(7):3308-3314. doi: 10.1111/jcmm.13630. Epub 2018 Apr 17.
Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end-stage organ failure. Two clinical subsets of the SSc are accepted: limited cutaneous SSc (lc-SSc) and diffuse cutaneous SSc (dc-SSc). At present, the aetiology and pathogenesis of SSc remain obscure, and consequently, disease outcome is unpredictable. Numerous studies suggest that reactive oxidizing species (ROS) play an important role in the pathogenesis of scleroderma. Over the years, several reports have supported this hypothesis for both lc-SSc and dc-SSc, although the specific role of oxidative stress in the pathogenesis of vascular injury and fibrosis remains to be clarified. The aim of the present review was to report and comment the recent findings regarding the involvement and role of oxidative stress in SSc pathogenesis. Biomarkers proving the link between ROS and the main pathological features of SSc have been summarized.
系统性硬化症(SSc)是一种罕见的结缔组织疾病,其特征为皮肤、骨骼肌肉和内脏器官的纤维化。其他表现包括免疫系统的激活和血管损伤。SSc 会导致终末期器官衰竭而致残和死亡。目前公认 SSc 有两种临床亚型:局限性皮肤型 SSc(lc-SSc)和弥漫性皮肤型 SSc(dc-SSc)。SSc 的病因和发病机制仍不清楚,因此疾病预后不可预测。许多研究表明,活性氧化物质(ROS)在硬皮病的发病机制中起重要作用。多年来,有几项报告支持这一假说,适用于 lc-SSc 和 dc-SSc,尽管氧化应激在血管损伤和纤维化发病机制中的具体作用仍有待阐明。本综述的目的是报告和评论最近关于氧化应激在 SSc 发病机制中的作用的研究结果。总结了证明 ROS 与 SSc 主要病理特征之间关联的生物标志物。