文献简介

出版社:国际皮肤性病学杂志

作  者:刘超凡;鲁静浩;周行;朱鹭冰;李明

编  号:

关键字:硬皮病,系统性;外周血管疾病;内皮细胞;干细胞;血小板

年  份:2017   点击量:140

文献摘要

【摘要】系统性硬皮病是以皮肤硬化和内脏器官纤维化为主要表现的自身免疫病,病因与发病机制不明,病理基础为血管病变、免疫异常激活及组织纤维化等。越来越多的研究表明,血管病变可能是系统性硬皮病发病的始发事件,包括小血管痉挛、内膜增生和血管减少等。概述近年来系统性硬皮病相关血管病变的研究进展,包括促血管新生因子(如血管内皮生长因子、基质细胞衍生因子1)和抗血管新生因子(如内皮抑素、正五聚蛋白3)及其受体(如血管内皮生长因子受体和尿激酶型纤溶酶原激活物受体)的紊乱、血管内皮祖细胞和血管内皮细胞数量和功能的异常、血管紧张性的调节失衡以及血小板及其释放物的功能异常等方面。

AbstractSystemic sclerosis(SSc) is an autoimmune disorder characterized by skin sclerosis and

internal organ fibrosis. Although its etiology and pathogenesis are unclear, the main pathological features of SSc are vasculopathy, aberrant immune activation and tissue fibrosis. Increasing studies have indicated that vasculopathy may be an initial event in the occurrence of SSc including small vessel spasm, intimal thickening, reduction of vessel number and so onThis review summarizes recent research progress in SSc-related vasculopathy, including the disarrangement of pro-angiogenic factors (e.g. vascular endothelial growth factor and stromal cell-derived factor-1), angiostatic factors(e.g. endostatin and pentraxin 3) and their receptors(e.g. Vascular endothelial growth factor receptor and urokinase-type plasminogen activator receptor), aberrant number and function of vascular endothelial progenitor cells and vascular endothelial cells, imbalance in the regulation of vascular tone, and dysfunction of platelets and their releasers in SSc