Systemic sclerosis (SSc) is a chronic, autoimmune, systemic, potentially lethal disease that involves the skin and internal organs. A complex interaction of genetic predisposition and environmental factors seems to trigger a wide range of vascular, immunological, and fibrotic changes. These changes represent the three cardinal features of SSc. Vascular changes seem to occur early in the disease and it involves endothelial cell activation/toxicity and other changes that involve angiogenic and angiostatic mechanisms. Different aspects of the immune system are involved including the humeral and cell-mediated immunity; they interact in a very complex manner and work together with the vascular changes in the formation of fibrosis, the end stage of SSc. © 2014 Elsevier Inc. All rights reserved.
Borazan, N. H., & Furst, D. E. (2013). Systemic Sclerosis, Scleroderma. In The Autoimmune Diseases: Fifth Edition (pp. 463–480). Elsevier Inc. https://doi.org/10.1016/B978-0-12-384929-8.00033-2