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This article is part of the supplement: Advances in systemic sclerosis and related fibrotic and vascular conditions

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How does endothelial cell injury start? The role of endothelin in systemic sclerosis

David Abraham1* and Oliver Distler2

Author Affiliations

1 Department of Medicine, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital and University College, Rowland Hill Street, London, NW3 2PF, UK

2 Center of Experimental Rheumatology and Department of Rheumatology, University Hospital Zürich, Gloriastrasse, CH-8032 Zürich, Switzerland

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Arthritis Research & Therapy 2007, 9(Suppl 2):S2  doi:10.1186/ar2186

Published: 15 August 2007


A considerable amount of research time has been invested in studies aimed at elucidating pathogenic processes in systemic sclerosis (SSc). Despite this, major challenges for biomedical science remain, such as identification of the key factors that determine susceptibility to SSc, and elucidation of the precise nature of the initiating event that causes endothelial cell injury and ultimately brings about the biological cascade(s) that lead to the pathologic vascular changes. Involved factors are likely to include genetic perturbations, environmental cues, tissue injury, infection and hypoxia/oxidative stress. As important as determining the initiating events are the identification and characterization of key factors that are functionally important in driving vascular disease progression, because these factors are potential targets for therapeutic intervention. This article reviews the role of endothelin as an example of a pleiotropic mediator with effects on various aspects of SSc pathogenesis, such as inflammation, vasculopathy and tissue remodelling.