The pathology of scleroderma vascular disease.
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.
Fleming
Jo Nadine
JN
Department of Pathology, 815 Mercer Street, Room 421, Brotman Building, Box 358050, University of Washington, Seattle WA 98109-4717, USA.
Schwartz
Stephen Mark
SM
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*
D020533:Angiogenesis Inhibitors