Effect of ambrisentan on peripheral circulation in patients with systemic sclerosis

Systemic sclerosis (SSc) is characterized by disturbed blood circulation. The effect of ambrisentan, an endothelin-A receptor-selective antagonist, on impaired peripheral circulation in SSc remains largely elusive. Here we show SSc patients, whose clinical symptoms such as cyanosis and Raynaud's phenomenon, were ameliorated by the treatment with ambrisentan. Additionally, objective evaluations with thermography showed improvement of hand coldness in steady-state and cold challenge tests. Ambrisentan might have a potential to improve peripheral circulation in SSc.

[1]  R. Ewert,et al.  Dual ETA/ETB vs. selective ETA endothelin receptor antagonism in patients with pulmonary hypertension , 2006 .

[2]  R. Dixon,et al.  Attenuation of pulmonary vascular hypertension and cardiac hypertrophy with sitaxsentan sodium, an orally active ET(A) receptor antagonist. , 2000, Pulmonary pharmacology & therapeutics.

[3]  J. Distler,et al.  Vascular alterations upon activation of TGFβ signaling in fibroblasts - implications for systemic sclerosis , 2010, Arthritis research & therapy.

[4]  H. Collard,et al.  Treatment of Idiopathic Pulmonary Fibrosis With Ambrisentan , 2013, Annals of Internal Medicine.

[5]  M. Mayes,et al.  Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. , 2004, Arthritis and rheumatism.

[6]  M. Jayson,et al.  Measurement of cold challenge responses in primary Raynaud's phenomenon and Raynaud's phenomenon associated with systemic sclerosis. , 1992, Annals of the rheumatic diseases.

[7]  F. Abtin,et al.  Brief report: effect of ambrisentan treatment on exercise-induced pulmonary hypertension in systemic sclerosis: a prospective single-center, open-label pilot study. , 2012, Arthritis and rheumatism.

[8]  Artemis-Ipf Investigators Treatment of idiopathic pulmonary fibrosis with Ambrisentan: A parallel, randomized trial , 2013 .

[9]  F. Ruschitzka,et al.  Endothelin receptor antagonists in congestive heart failure: a new therapeutic principle for the future? , 2001, Journal of the American College of Cardiology.

[10]  R. Madhok,et al.  Circulating endothelin-1 levels in systemic sclerosis subsets--a marker of fibrosis or vascular dysfunction? , 1996, Journal of Rheumatology.

[11]  M. Mayes,et al.  Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial , 2010, Annals of the rheumatic diseases.

[12]  W. Kirch,et al.  Inhibition of endothelin receptors in the treatment of pulmonary arterial hypertension: does selectivity matter? , 2008, European heart journal.

[13]  S. Oparil,et al.  Endothelin-receptor antagonist bosentan prevents and reverses hypoxic pulmonary hypertension in rats. , 1995, Journal of applied physiology.

[14]  M. Bruzzone,et al.  Efficacy of ambrisentan in the treatment of digital ulcers in patients with systemic sclerosis: a preliminary study. , 2013, Rheumatology.

[15]  W. Seeger,et al.  Safety and tolerability of bosentan in idiopathic pulmonary fibrosis: an open label study , 2007, European Respiratory Journal.

[16]  C. Black,et al.  Circulating endothelin-1 levels in systemic sclerosis subsets--a marker of fibrosis or vascular dysfunction? , 1994, The Journal of rheumatology.

[17]  W. Seeger,et al.  Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. , 1999, American journal of respiratory and critical care medicine.

[18]  D. Kohan,et al.  Frequency of edema in patients with pulmonary arterial hypertension receiving ambrisentan. , 2012, The American journal of cardiology.

[19]  F. Fedele,et al.  Safety and efficacy evaluation of ambrisentan in pulmonary hypertension , 2012, Expert opinion on drug safety.

[20]  Y. Asano Future treatments in systemic sclerosis , 2010, The Journal of dermatology.