Endothelin and Systemic Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Endothelin and Systemic Scleroderma Endothelin Receptor Antagonists

Endothelin and Systemic Scleroderma

Endothelin is a protein that helps to regulate contraction and relaxation of the smooth muscle found in the walls of blood vessels and internal organs (except the heart). An overproduction of endothelin, whether by illness or medication, can cause blood vessels to constrict and blood pressure to increase. In scleroderma, it can cause pulmonary arterial hypertension (PAH). ISN. (Also see Causes of Scleroderma, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)

Endothelin. Endothelins are protein that constrict blood vessels and raise blood pressure. Among the strongest vasoconstrictors known, endothelins are implicated in vascular diseases of several organ systems, including the heart, general circulation and brain.

Study Finds Cell Microparticles Correlated with Lung Function in Systemic Sclerosis (SSc) Patients. Investigating microparticles further may help to better understand the pathogenesis of disease and enhance disease activity monitoring in patients with SSc. Scleroderma News, 08/17/2015.

Increased expression of chemerin in endothelial cells due to Fli1 deficiency may contribute to the development of digital ulcers in systemic sclerosis. Increased chemerin expression in dermal blood vessels may be associated with the development of digital ulcers in SSc. PubMed, 12/23/2014.

Impaired in vivo neovascularization capacity of endothelial progenitor cells (EPC) in patients with systemic sclerosis (SSc). EPCs contribute to vascularization by being incorporated into vessel walls and by differentiating into endothelial cells and these EPC functions are impaired in SSc patients. PubMed, Arthritis Rheum, 2013 Dec 24.

Endothelin Receptor Antagonist (ETRA)

Effect of endothelin–1 receptor antagonists (ETRAs) on skin fibrosis in scleroderma (SSc) patients from the EUSTAR database. ETRAs have well–proven clinical effects in the prevention of digital ulcers and in the treatment of pulmonary hypertension, but there is no evidence from clinical studies to support its use as an anti–fibrotic therapy. Journal of Scleroderma and Related Disorders, 03/30/2016. (Also see Skin Fibrosis)

Angiotensin receptor type 1 (AT1R) and endothelin receptor type A (ETAR) on immune cells mediate migration and the expression of IL-8 and CCL18 when stimulated by autoantibodies from systemic sclerosis (SSc) patients. PubMed, Arthritis Res Ther, 2014 Mar 11;16(2):R65. (Also see Antibodies in Systemic Scleroderma)

Oestradiol ameliorates monocrotaline pulmonary hypertension via NO, prostacyclin and endothelin-1 pathways. Oestrogen deficiency may aggravate development of pulmonary hypertension. ERJ May 1, 2013 vol. 41 no. 5 1116-1125.

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