title: The pathology of scleroderma vascular disease.
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.
authors.affiliation: Department of Pathology, 815 Mercer Street, Room 421, Brotman Building, Box 358050, University of Washington, Seattle WA 98109-4717, USA.
authors.forename: Jo Nadine
authors.initials: JN
authors.lastname: Fleming
authors.forename: Stephen Mark
authors.initials: SM
authors.lastname: Schwartz
mesh_terms: D020533:Angiogenesis Inhibitors / Q000502:physiology; D002196:Capillaries / Q000473:pathology; D006801:Humans; D012595:Scleroderma, Systemic / Q000473:pathology*; D017539:Tunica Intima / Q000473:pathology*; D014652:Vascular Diseases / Q000473:pathology*
chemical_list: D020533:Angiogenesis Inhibitors