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Overview of pathogenesis of systemic sclerosis

Abraham, DJ; Krieg, T; Distler, J; Distler, O; (2009) Overview of pathogenesis of systemic sclerosis. RHEUMATOLOGY , 48 3 - 7. 10.1093/rheumatology/ken481.

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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.

Type: Article
Title: Overview of pathogenesis of systemic sclerosis
Location: Athens, GREECE
DOI: 10.1093/rheumatology/ken481
Keywords: Endothelial cell, Endothelin-1, Vasculopathy, Fibrosis, Scleroderma, ANTIENDOTHELIAL CELL ANTIBODIES, ENDOTHELIAL GROWTH-FACTOR, GENE-EXPRESSION, SCLERODERMA FIBROBLASTS, MICROVASCULAR PERICYTES, MESENCHYMAL TRANSITION, PULMONARY-HYPERTENSION, EXTRACELLULAR-MATRIX, SMOOTH-MUSCLE, SKIN
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: http://discovery.ucl.ac.uk/id/eprint/168693
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