系统性硬化症病理生理学中的细胞和分子机制

T. Hua-Huy, A.T. Dinh-Xuan
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引用次数: 21

摘要

纤维化的特点是胶原和其他细胞外基质物质不成比例地积聚,导致器官功能障碍和衰竭。在系统性硬化症中,参与纤维化病理生理的细胞和分子机制是高度复杂的,但很少被理解。解剖病理结果显示斑片状炎症细胞浸润、微血管损伤和纤维化灶并存。最普遍接受的假设之一认为内皮细胞激活是诱发炎症和自身免疫激活的触发现象。由此产生的细胞因子和自身抗体加速了正常成纤维细胞的增殖速度,并加速了它们向肌成纤维细胞的转化,导致弥漫性纤维化。本文综述了系统性硬化症纤维形成的细胞和分子机制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cellular and molecular mechanisms in the pathophysiology of systemic sclerosis

Fibrosis is characterized by disproportionate accumulation of collagens and other extracellular matrix substances, resulting in organ dysfunction and failure. In systemic sclerosis, cellular and molecular mechanisms involved in the pathophysiology of fibrosis are highly complex and yet barely understood. Anatomopathological findings showed the coexistence of patchy inflammatory cell infiltration, microvascular injuries, and fibrotic foci. One of the most commonly accepted hypotheses considers endothelial activation as the triggering phenomenon inducing inflammatory and autoimmunity activation. The resulting cytokines and autoantibodies production accelerates the proliferating rate of normal fibroblasts and their transformation into myofibroblasts, leading to diffuse fibrosis. This review aims to focus on cellular and molecular mechanisms implicated in the fibrogenesis of systemic sclerosis.

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来源期刊
Pathologie-biologie
Pathologie-biologie 医学-病理学
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6-12 weeks
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