Overview of pathogenesis of systemic sclerosis
Overview of pathogenesis of systemic sclerosis
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DOI：
10.1093/rheumatology/ken481
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发表时间：
2009-06-01
期刊：
影响因子：
5.5
通讯作者：
Distler, O.
中科院分区：
文献类型：
--
作者：
Abraham, D. J.;Krieg, T.;Distler, O.
SSc的病因正在进行研究，因为这种疾病发展的确切事件仍不清楚。已知发病机制涉及内皮、上皮、成纤维细胞、先天性和适应性免疫系统及其组分免疫介质。内皮细胞损伤可能是引发因素，但确切的触发事件仍然难以捉摸。血管生成似乎也失调。血管病变在不同器官中表现出相似性（例如肺动脉高压、肾脏疾病、指尖溃疡）。内皮素-1是血管病变的有效介质，因此代表了SSc血管特征干预的高度相关靶点。
The aetiology of SSc is subject to ongoing research, as the precise events that underlie the development of this disease remain unclear. The pathogenesis is known to involve endothelium, epithelium, fibroblasts, innate and adaptive immune systems and their component immunological mediators. Endothelial cell damage may be the initiating factor, but the precise triggering event(s) remain elusive. Angiogenesis also appears to be dysregulated. Vasculopathy shows similarities in different organs (e.g. pulmonary arterial hypertension, renal disease, digital tip ulcers). Endothelin-1 is a potent mediator of vasculopathy, and hence represents a highly relevant target for intervention of vascular features in SSc.