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A series of genetic and epidemiological studies have demonstrated that\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c onset is determined by the accumulation of predisposing factors related to environmental influences, while genetic factors affect the susceptibility to and the severity of this disease. This notion has been confirmed by recent advance in animal models. The initial trigger of\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c is believed to be autoimmune attacks to endothelial cells, which occur in individuals with the genetic susceptibility to autoimmune diseases and\/or the cumulative exposure to certain\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c\u2010related environmental influences. Then, endothelial cells are aberrantly activated or damaged, leading to the development of vascular structural changes, such as destructive vasculopathy and proliferative obliterative vasculopathy, and tissue fibrosis. In parallel, inflammatory cells activate\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c fibroblasts and modify the metabolism of extracellular matrix by soluble factors and autoantibodies. Prior to or during these processes,\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c fibroblasts acquire the ability to selectively respond to profibrotic growth factors and cytokines, persistently producing excessive amount of extracellular matrix.\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c fibroblasts also modify immune responses, at least those of\n                    <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>\n                    4\n                    <jats:sup>+<\/jats:sup>\n                    T cells, in the microenvironment through the secretion of immune regulatory molecules. Thus, various types of individually activated cells interact with each other and coordinately drive an\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c\u2010specific disease cascade, leading to the development of unique clinical symptoms. This article provides an overview of the current understanding of the pathogenesis of\n                    <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\n                    c with the recent advance in the research field of this disease.\n                  <\/jats:p>","DOI":"10.1111\/1346-8138.14153","type":"journal-article","created":{"date-parts":[[2017,12,10]],"date-time":"2017-12-10T23:06:48Z","timestamp":1512947208000},"page":"128-138","source":"Crossref","is-referenced-by-count":128,"title":["Systemic sclerosis"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5560-9778","authenticated-orcid":false,"given":"Yoshihide","family":"Asano","sequence":"first","affiliation":[{"name":"Department of Dermatology University of Tokyo Graduate School of Medicine Tokyo 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