title: Endothelin-1 levels in scleroderma patients: a pilot study.
abstract: Endothelin-1 (ET-1) is a potent endogenous vasoconstrictor, which mediates vascular wall cells proliferation, fibrosis, and inflammation through two types of ET-1 receptors (ET-A and ET-B). In our retrospective study the serum levels of ET-1 in 18 systemic sclerosis (SSc) patients with and without digital ulcers (DUs) were assessed to observe possible correlation between the levels of ET-1, the evolution of SSc, and the therapy with an ET-1 antagonist (bosentan). In all our patients, the levels of ET-1 were found higher than normal range and correlate with the severity of the disease. Furthermore we also observed that in patients without DUs the levels of ET-1 were higher and did not correlate with new DUs development. In conclusion, the levels of ET-1 in our studied patients do not correlate with the possible development of DUs. The reduction of ET-1 levels in DUs patients in therapy with bosentan confirms the efficacy of this molecule both for treatment and prevention of digital ulcers. The inhibition of ET-A receptor by its antagonist may activate the opposite ET-B receptors, with well-known function ET-1 degradation and reducing of ET-1 serum level as confirmed in our pilot study.
authors: DISSAL, Section of Dermatology, IRCCS University Hospital San Martino-IST, 16132 Genoa, Italy.
authors: Emanuele
authors: E
authors: Cozzani
authors: Sanja
authors: S
authors: Javor
authors: Erika
authors: E
authors: Laborai
authors: Massimo
authors: M
authors: Drosera
authors: Aurora
authors: A
authors: Parodi
references: Arthritis Rheum. 2004 Dec;50(12):3985-93
references: 15593188
references: Dermatologica. 1991;183(1):15-20
references: 1769409
references: Int Angiol. 2011 Oct;30(5):493-5
references: 21804491
references: Acta Derm Venereol. 2011 Oct;91(6):716-7
references: 21879251
references: J Rheumatol. 2009 Jun;36(6):1235-9
references: 19369451
references: Arthritis Rheum. 1995 Mar;38(3):351-60
references: 7880189
references: J Dermatol. 2012 Jan;39(1):48-51
references: 21955007
references: Best Pract Res Clin Rheumatol. 2012 Feb;26(1):13-24
references: 22424190
references: Rheum Dis Clin North Am. 2003 May;29(2):293-313
references: 12841296