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International Journal of Rheumatology
Volume 2011, Article ID 201787, 7 pages
http://dx.doi.org/10.1155/2011/201787
Review Article

Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis

Division of Immunology and Rheumatology, Departments of Dermatology and Medicine, Stanford University School of Medicine, Palo Alto VA Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94304, USA

Received 2 June 2011; Accepted 17 August 2011

Academic Editor: Virginia D. Steen

Copyright © 2011 Kait Arefiev et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.