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Rheumatology 2009 48(Supplement 3):iii3-iii7; doi:10.1093/rheumatology/ken481
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

This article appears in the following Rheumatology issue: Ten years of partnership: translating ideas into progress in systemic sclerosis [View the issue table of contents]

Reviews

Overview of pathogenesis of systemic sclerosis

D. J. Abraham1, T. Krieg2, J. Distler3 and O. Distler4

1Centre for Rheumatology and Connective Tissue Disease, Royal Free and University College Medical School, University College London, London, UK, 2Department of Dermatology, University of Cologne, Cologne, 3Department for Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany and 4Rheumaklinik und Institut fuer physikalische Medizin, University of Zurich, Zurich, Switzerland.

Correspondence to: D. J. Abraham, Centre for Rheumatology and Connective Tissue Disease, Royal Free and University College Medical School, University College London, Hampstead Campus, Rowland Hill Street, Hampstead, London NW3 2PF, UK. E-mail: d.abraham{at}medsch.ucl.ac.uk


  Abstract

The aetiology of SSc is subject to ongoing research, as the precise events that underlie the development of this disease remain unclear. The pathogenesis is known to involve endothelium, epithelium, fibroblasts, innate and adaptive immune systems and their component immunological mediators. Endothelial cell damage may be the initiating factor, but the precise triggering event(s) remain elusive. Angiogenesis also appears to be dysregulated. Vasculopathy shows similarities in different organs (e.g. pulmonary arterial hypertension, renal disease, digital tip ulcers). Endothelin-1 is a potent mediator of vasculopathy, and hence represents a highly relevant target for intervention of vascular features in SSc.

KEY WORDS: Endothelial cell, Endothelin-1, Vasculopathy, Fibrosis, Scleroderma

Submitted 30 January 2008; revised version accepted 1 December 2008.
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