Hua-Huy T, Dinh-Xuan A T
UPRES-EA 2511, laboratoire de physiologie respiratoire, hôpital Cochin-Paris Centre, Assistance publique-Hôpitaux de Paris, université Paris-Descartes, Sorbonne Paris-Cité, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
UPRES-EA 2511, laboratoire de physiologie respiratoire, hôpital Cochin-Paris Centre, Assistance publique-Hôpitaux de Paris, université Paris-Descartes, Sorbonne Paris-Cité, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
Pathol Biol (Paris). 2015 Apr;63(2):61-8. doi: 10.1016/j.patbio.2015.03.003. Epub 2015 Mar 25.
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.
纤维化的特征是胶原蛋白和其他细胞外基质物质过度积累，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过程的细胞和分子机制高度复杂，然而人们对此却知之甚少。解剖病理学研究结果显示，存在散在性炎性细胞浸润、微血管损伤和纤维化病灶。最被广泛接受的假说之一认为，内皮细胞激活是引发炎症和自身免疫激活的现象。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率及其向肌成纤维细胞的转化，导致弥漫性纤维化。本综述旨在聚焦于系统性硬化症纤维化形成过程中涉及的细胞和分子机制。
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