Endothelin receptor antagonism--new perspectives in the treatment of systemic sclerosis.

Endothelin-1 is a naturally occurring polypeptide which possesses a broad range of activities including vasospastic, proinflammatory and profibrotic properties. Systemic sclerosis is a multisystem connective tissue disease characterized by vascular damage, inflammatory infiltrates and progressive fibrosis of the skin and internal organs. The results of the recent studies indicate that endothelin-1 may be a key element of the pathogenesis of systemic sclerosis. Accordingly, new class of drugs, endothelin receptor antagonists have been introduced for treatment of patients with systemic sclerosis. This article reviews the role of endothelin-1 in the pathogenesis of systemic sclerosis and the implications of endothelin receptor antagonism in the treatment of systemic sclerosis.