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.

Inhibition of EZH2 prevents fibrosis and restores normal angiogenesis in scleroderma (SSc). . Our results demonstrate that overexpression of EZH2 in SSc fibroblasts and endothelial cells is profibrotic and antiangiogenic. PubMed, Proc Natl Acad Sci U S A, 02/12/2019.

Epithelial Fli1 deficiency drives systemic autoimmunity and fibrosis: Possible roles in scleroderma. This study uncovers unidentified roles of dysregulated epithelial cells in SSc pathogenesis. PubMed, J Exp Med, 2017 Apr 3;214(4):1129-1151.

Proangiogenic effects of soluble α-Klotho (sKl) on systemic sclerosis (SSc) dermal microvascular endothelial cells (MVEC) Our findings provide the first evidence that α-Klotho is significantly decreased in the microvasculature in SSc skin and that sKl administration may effectively improve SSc–MVEC functions in vitro by acting as a powerful proangiogenic factor. BioMed Central, Arthritis Research & Therapy, 02/10/2017.

Endothelin Receptor Antagonist (ETRA)

Anti–Angiotensin II Type 1 Receptor and Anti–Endothelial Cell Antibodies: A Cross–Sectional Analysis of Pathological Findings in Allograft Biopsies. The data show an association between non-HLA antibodies detected in the ECXM and AT1R ELISA and microvascular injury observed in antibody mediated rejection. PubMed, Transplantation, 2017 Mar;101(3):608-615. (Also see Antibodies in Systemic Scleroderma)

Go to Causes of Scleroderma: Environmental

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