Abstract |
Systemic sclerosis is characterized by three distinct pathologic processes: fibrosis, cellular/humoral autoimmunity, and specific vascular changes. Although a mild vasculitis may sometimes be present, the vascular pathology of scleroderma is not necessarily inflammatory and is best characterized as a vasculopathy. In this article, the authors propose that SSc vasculopathy is the result of an early event involving vascular injury that eventuates in a vicious cycle mediated in part by the immune process. The subsequent vascular malformation and rarefaction may be a function of systemic angiogenic dysregulation, with over expression of vascular endothelial growth factor but a lack of proper interactions with smooth muscle cells needed to stabilize and organize blood vessels.
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Authors | Jo Nadine Fleming, Stephen Mark Schwartz |
Journal | Rheumatic diseases clinics of North America
(Rheum Dis Clin North Am)
Vol. 34
Issue 1
Pg. 41-55; vi
(Feb 2008)
ISSN: 0889-857X [Print] United States |
PMID | 18329531
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Chemical References |
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Topics |
- Angiogenesis Inhibitors
(physiology)
- Capillaries
(pathology)
- Humans
- Scleroderma, Systemic
(pathology)
- Tunica Intima
(pathology)
- Vascular Diseases
(pathology)
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