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Published on: October 22, 2014
Endothelin and collagen vascular disease: a review with special reference to Raynaud's phenomenon and systemic
1Department of Internal Medicine, University of Tsukuba.
Insights
Endothelin-1 (ET-1) is a potent vasoconstrictor linked to vascular diseases. Elevated ET-1 levels in systemic sclerosis and Raynaud
Area of Science:
- Vascular Biology
- Endocrinology
- Rheumatology
Background:
- Endothelin-1 (ET-1) is a potent vasoconstrictor involved in vascular regulation.
- Elevated ET-1 levels are observed in various diseases with vascular involvement and endothelial cell activation.
- Diseases implicated include systemic sclerosis, Raynaud's phenomenon, pulmonary hypertension, Buerger's disease, and Takayasu's arteritis.
Purpose of the Study:
- To review the clinical significance and pathogenetic role of endothelin in collagen vascular diseases.
- To focus on the role of ET-1 in Raynaud's phenomenon and systemic sclerosis.
Main Methods:
- Review of recent findings on endothelin in collagen vascular diseases.
- Analysis of clinical and laboratory observations supporting vascular pathogenesis in systemic sclerosis.
Main Results:
- Plasma ET-1 levels are elevated in patients with systemic sclerosis.
- Higher ET-1 elevation is noted in diffuse scleroderma compared to limited scleroderma patients.
- These findings support a vascular pathogenesis for systemic sclerosis.
Conclusions:
- Circulating ET-1 plays a significant role in the pathogenesis of vascular and fibrotic lesions in collagen vascular diseases.
- The elevated ET-1 levels in systemic sclerosis further support its vascular etiology.
Abstract:
Endothelin-1 (ET-1), endothelin-2, and endothelin-3, a family of 21-aminoacid peptides, are potent, long-acting vasoconstrictors of mammalian blood vessels. Elevated levels of ET-1 have been found in the circulation of patients with various diseases, all of which have some degree of vascular involvement that is associated with endothelial cell activation. These diseases include systemic sclerosis, primary and secondary Raynaud's phenomenon, pulmonary hypertension, Buerger's disease, and Takayasu's arteritis. The role of circulating ET-1 in the pathogenesis of the vascular and fibrotic lesions in these conditions remains to be elucidated. This review focuses on the recent findings of the clinical significance and pathogenetic role of endothelin in collagen vascular diseases, especially in Raynaud's phenomenon and systemic sclerosis. Although the etiology of systemic sclerosis is unknown, a vascular pathogenesis is supported by a variety of clinical and laboratory observations. The fact that plasma levels of ET-1 are elevated in patients with systemic sclerosis and this elevation is more remarkable in patients with diffuse scleroderma compared with limited scleroderma patients might further support the vascular pathogenesis of systemic sclerosis.
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