|Endothelin and Systemic Scleroderma||Endothelin Receptor Antagonists|
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. Answers.com.
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.
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.
Reading Voices of Scleroderma Books: Diana Kramer.
Sharing Scleroderma Awareness Bracelets: Deb Martin, Brenda Miller, Vickie Risner.
Thanks to UNITED WAY donors of Central New Mexico and Snohomish County!
Patricia Ann Black: Marilyn Currier, Shelley Ensz, Richard Howitt, Gerald and Pat Ivanejko, Juno Beach Condo Association, Keith and Rosalyn Miller, and Elaine Wible.
Gayle Hedlin: Daniel and Joann Pepper and Nancy Smithberg.
Janet Paulmenn: Anonymous, Mary Jo Austin, Shelley Blaser, Susan Book, Dennis and Pat Clayton, Grace Cunha, Cindy Dorio, Michael and Patricia Donahue, Shelley Ensz, Nancy Falkenhagen, Jo Frowde, Alice Gigl, Margaret Hollywood, Karen Khalaf and Family, Susan Kvarantan, Bradley Lawrence, Jillyan Little, Donna Madge, Michele Maxson, Barry and Judith McCabe, John Moffett, My Tribute Foundation, Joan-Marie Permison, John Roberts, Margaret Roof, Maryellen Ryan, Mayalin and Kiralee Murphy, Nancy Settle-Murphy, and Bruce and Elizabeth Winter.
SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:
International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA
Email [email protected] to request our Welcome email, or to report bad links or to update this page content.