Kähäri V M
Department of Dermatology, University of Turku, Finland.
Ann Med. 1993 Dec;25(6):511-8.
Systemic scleroderma is an acquired disorder which typically results in fibrosis of the skin and internal organs. The pathogenesis of systemic scleroderma is characterized by three distinct processes: microvascular alterations including capillary endothelial cell injury, perivascular inflammatory reaction in dermis, and excessive accumulation of collagen in the dermal layer of lesional skin. In this review, molecular mechanisms resulting in activation of collagen synthesis by dermal fibroblasts in scleroderma are discussed. Specifically, the role of inflammatory cells and the cytokines/growth factors produced by these cells in the pathogenesis of scleroderma is emphasized. The possibilities for prevention and resolution of tissue fibrosis on the basis of these observations are also discussed. Understanding the pathogenetic mechanisms of scleroderma at a molecular level is likely to provide possibilities for development of more specific therapeutic modalities for this and other fibrotic disorders.
系统性硬化症是一种后天性疾病，通常会导致皮肤和内脏器官纤维化。系统性硬化症的发病机制具有三个不同的过程：微血管改变，包括毛细血管内皮细胞损伤、真皮层血管周围炎症反应以及病变皮肤真皮层中胶原蛋白的过度积累。在这篇综述中，讨论了导致硬皮病中真皮成纤维细胞激活胶原蛋白合成的分子机制。具体而言，强调了炎症细胞以及这些细胞产生的细胞因子/生长因子在硬皮病发病机制中的作用。还讨论了基于这些观察结果预防和解决组织纤维化的可能性。在分子水平上理解硬皮病的发病机制可能为开发针对这种疾病和其他纤维化疾病的更特异性治疗方法提供可能性。
