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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.

Increased expression of aquaporin-1 (AQP1) in dermal fibroblasts and dermal microvascular endothelial cells possibly contributes to skin fibrosis and edema in patients with systemic sclerosis (SSC). AQP1 is up-regulated in SSc dermal fibroblasts and SSc endothelial cells at least partially due to autocrine TGF-ß stimulation and Fli1 deficiency PubMed, J Dermatol Sci, 2019 Jan;93(1):24-32.

Endothelin Receptor Antagonist (ETRA)

Prevalence of Angiotensin II Type 1 Receptor Antibodies (AT1RAb) in Persons With Hypertension and Relation to Blood Pressure (BP) and Medication. AT1RAb was prevalent among hypertensive adults and was associated with higher BP among persons on anangiotensin receptor blocker. PubMed, Am J Hypertens, 2020 Aug 4;33(8):734-740. (Also see Antibodies in Systemic Scleroderma)

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