Abraham D J, Krieg T, Distler J, Distler O
Centre for Rheumatology and Connective Tissue Disease, Royal Free and University College Medical School, University College London, Hampstead, London, UK.
Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii3-7. doi: 10.1093/rheumatology/ken481.
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.
系统性硬化症的病因仍在研究中，因为导致这种疾病发生的确切机制尚不清楚。已知其发病机制涉及内皮细胞、上皮细胞、成纤维细胞、固有免疫和适应性免疫系统及其组成的免疫介质。内皮细胞损伤可能是起始因素，但确切的触发事件仍不明确。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（如肺动脉高压、肾脏疾病、指尖溃疡）。内皮素-1是血管病变的一种强效介质，因此是系统性硬化症血管特征干预的一个高度相关靶点。
