Article
[Systemic sclerosis: pathophysiology of a multifaceted disease].
Presse medicale (Paris, France : 1983) - 1 Dec 2006
Servettaz Amélie, Agard Christian, Tamby Mathieu C, Guilpain Philippe, Guillevin Loïc, Mouthon Luc
Abstract excerpt
Systemic sclerosis is a rare disease characterized by vascular hyperreactivity and collagen deposition. Endothelial cell, fibroblast and lymphocyte abnormalities have been reported in systemic sclerosis. Fibroblast dysfunction is characterized by uncontrolled activation of the transforming growth factor-beta (TGF-beta) pathway and excess synthesis of both connective tissue growth factor (CTGF) and free radicals....
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