Which medications in the drug class Endothelin receptor antagonist are used in the treatment of Scleroderma?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Endothelin receptor antagonist

These agents bind to endothelin receptors present in endothelium and vascular smooth muscle cells. The effect can result in vasodilation.

Bosentan (Tracleer)

Bosentan is a dual endothelin A and B receptor antagonist for treatment of pulmonary arterial hypertension. It decreases both pulmonary and systemic vascular resistance and increases cardiac output without increasing heart rate

Ambrisentan (Letairis)

Ambrisentan is an endothelin receptor antagonist indicated for pulmonary arterial hypertension in patients with World Health Organization (WHO) class II or III symptoms. It improves exercise ability and decreases progression of clinical symptoms. Ambrisentan inhibits vessel constriction and elevation of blood pressure by competitively binding to endothelin-1 receptors ETA and ETB in endothelium and vascular smooth muscle. This leads to significant increase in cardiac index associated with significant reduction in pulmonary artery pressure, pulmonary vascular resistance, and mean right atrial pressure.

Because of the risks of hepatic injury and teratogenic potential, this agent is available only through the Letairis Education and Access Program (LEAP). Prescribers and pharmacies must register with LEAP in order to prescribe and dispense. For more information, see http://www.letairis.com or call (866) 664-LEAP (5327).

Macitentan (Opsumit)

Macitentan is a novel agent acting as dual endothelin receptor-1(ETA and ETB) antagonist with sustained receptor-binding properties. It has been approved by the FDA for the treatment of pulmonary hypertension. Studies assessing its efficacy and safety in ischemic digital ulcers secondary to Raynaud phenomenon is systemic sclerosis are under way.

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