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: 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)

Chemical References

• Angiogenesis Inhibitors

Topics

• Angiogenesis Inhibitors (physiology)
• Capillaries (pathology)
• Humans
• Scleroderma, Systemic (pathology)
• Tunica Intima (pathology)
• Vascular Diseases (pathology)