Abstract
In April 2006, 30 international experts gathered in Athens, Greece, to discuss systemic sclerosis (SSc) and related connective tissue diseases (CTDs). SSc is a clinically heterogeneous and complex disease that is characterized by vascular dysfunction, vascular and extravascular fibrosis, and characteristic immune derangements, and for which few treatment options are available. The aims of the CTD International Scientific Advisory Board were threefold: to define the role of local mediators in CTDs, in particular to identify the nature of the initial insult in CTDs and to consider the role of genetic perturbations in CTDs; to translate what has been learned from clinical trials into clinical practice and to evaluate current treatment options for CTDs and their complications; and to address future directions for the management of CTDs and associated rare diseases, based on the biologic mechanisms elucidated. This supplement provides a comprehensive review of the 2-day meeting in order to share important insights, opinions, and clinical approaches to management of CTDs. The first paper in this supplement, that by Abraham and Distler [1], reviews the potential role of endothelin (ET)-1 and the crosstalk and interplay between the various inflammatory mediators that lead to the vascular changes characteristic of the pathophysiology of SSc and other CTDs. The authors present the available evidence suggesting that ET-1 may play a key role at the early stages of CTDs by activating fibroblasts and pericytes. As yet, the event triggering endothelial cell injury is unknown.
Highlights
The first paper in this supplement, that by Abraham and Distler [1], reviews the potential role of endothelin (ET)-1 and the crosstalk and interplay between the various inflammatory mediators that lead to the vascular changes characteristic of the pathophysiology of systemic sclerosis (SSc) and other connective tissue diseases (CTDs)
Having established that there is a need to improve our understanding of the factors that potentially initiate endothelial injury, Koch and Distler [2] review the possible initiators of vascular injury in CTDs, focusing on dysregulation of angiogenesis as an important process in the pathogenesis of
This inflammatory activity is believed to contribute to the increased risk for cardiovascular morbidity and mortality in rheumatoid arthritis and to the accelerated atherosclerosis seen in systemic lupus erythematosus
Summary
The first paper in this supplement, that by Abraham and Distler [1], reviews the potential role of endothelin (ET)-1 and the crosstalk and interplay between the various inflammatory mediators that lead to the vascular changes characteristic of the pathophysiology of SSc and other CTDs. The authors present the available evidence suggesting that ET-1 may play a key role at the early stages of CTDs by activating fibroblasts and pericytes. Mayes and Trojanowska review recent evidence for the contribution of a candidate gene, Fli1, whose dysregulation appears to play a pathologic role in SSc skin fibrosis as well as having an effect on vessel degeneration.
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