Department of Biology, Memphis University School, Memphis, TN 38119, USA.
Division of Rheumatology, University of Tennessee Health Sciences Center, Memphis, TN 38163, USA.
Int J Mol Sci. 2023 Sep 21;24(18):14385. doi: 10.3390/ijms241814385.
Systemic sclerosis, commonly known as scleroderma, is an autoimmune disorder characterized by vascular abnormalities, autoimmunity, and multiorgan fibrosis. The exact etiology is not known but believed to be triggered by environmental agents in a genetically susceptible host. Vascular symptoms such as the Raynaud phenomenon often precede other fibrotic manifestations such as skin thickening indicating that vascular dysfunction is the primary event. Endothelial damage and activation occur early, possibly triggered by various infectious agents and autoantibodies. Endothelial dysfunction, along with defects in endothelial progenitor cells, leads to defective angiogenesis and vasculogenesis. Endothelial to mesenchymal cell transformation is another seminal event during pathogenesis that progresses to tissue fibrosis. The goal of the review is to discuss the molecular aspect of the endothelial dysfunction that leads to the development of systemic sclerosis.
系统性硬化症，俗称硬皮病，是一种自身免疫性疾病，其特征为血管异常、自身免疫和多器官纤维化。确切病因尚不清楚，但被认为是在遗传易感宿主中由环境因素引发。血管症状，如雷诺现象，通常先于其他纤维化表现，如皮肤增厚，表明血管功能障碍是主要事件。内皮损伤和激活发生较早，可能由各种感染因子和自身抗体触发。内皮功能障碍以及内皮祖细胞缺陷导致血管生成和血管发生缺陷。内皮细胞向间充质细胞转化是发病机制中的另一个重要事件，导致组织纤维化。本文的目的是讨论导致系统性硬化症发生的内皮功能障碍的分子机制。
