Endothelin

Abstract

ET-1 has various biological functions, including vasodilation/vasoconstriction, extracellular matrix production, and proliferation and migration on vascular endothelial cells, vascular smooth muscle cells, fibroblasts, and macrophages/immune cells. Experimental and clinical studies have highlighted the involvement of ET-1 in the pathogenesis of pulmonary arterial hypertension, vasculopathy, and fibrosis of the skin, lungs, and other organs in systemic sclerosis as well as the potential therapeutic role for endothelin antagonists in these conditions in systemic sclerosis. This chapter reviews the current knowledge of the endothelin/endothelin receptor system and focuses on the effects of ET-1 and its antagonism on vasculopathy and fibrosis in systemic sclerosis.