Scleroderma Types Systemic Symptoms Lungs PH Treatments

PAH/PH Treatments: Endothelin Receptor Antagonists

Overview of Endothelin Receptor Antagonists

"Time to clinical worsening was significantly faster with endothelin receptor antagonist."

Excess endothelin has been found in people with pulmonary hypertension, causing constriction in the blood vessels in the lungs. Endothelin receptor antagonists are designed to block the excess endothelin by various means.

SSc-PAH Outcomes Worse With Initial ERA. Time to clinical worsening was significantly faster with an endothelin receptor antagonist (ERA) as primary therapy for systemic sclerosis-related pulmonary arterial hypertension compared with a phosphodiesterase-5 (PDE5) inhibitor, or the combination of a PDE5 inhibitor and an ERA, researchers reported. MedPageToday.

Bosentan (Tracleer) by Actelion

Bosentan (Tracleer) by Actelion is proving useful for a variety of scleroderma symptoms, including pulmonary fibrosis, pulmonary hypertension, Raynaud's, and skin fibrosis. ISN.

Letairis (Ambrisentan) by Gilead

Letairis (Ambrisentan) by Gilead is an endothelin receptor antagonist for pulmonary hypertension. ISN.

Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure (mPAP) associated with systemic sclerosis. This is the first randomized, double-blind, placebo-controlled study testing the effect of ambrisentan in patients with mildly elevated mPAP and/or exercise pulmonary hypertension. PubMed, Arthritis Res Ther, 2019 Oct 26;21(1):217. (Also see Letairis (Ambrisentan) and Clinical Trials)

Macitentan (Opsumit) by Actelion

Macitentan (Opsumit) by Actelion is an oral dual endothelin receptor antagonist for pulmonary hypertension, which submitted its FDA approval application in October 2012. ISN.

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