Understanding of the function of endothelin-1 in the pathophysiology of endothelial disease, in particular pulmonary arterial hypertension (PAH), has paved the way for the development of endothelin-receptor antagonists (ERAs) and explains the leading role they now play in the treatment armamentarium for this disease.
Three active ERA drugs (bosentan, sitaxentan, ambrisentan) are currently approved for the treatment of PAH in France. Several randomised clinical trials have demonstrated their efficacy and safety in PAH.
Perspectives and conclusion
Besides its vasoconstrictor effect, endothelin-1 (ET-1) plays a pivotal role in cell proliferation and apoptosis. ERAs are innovative drugs potentially useful in some pulmonary disorders such as idiopathic pulmonary fibrosis or systemic sclerosis, even though the preliminary results published remain insufficient or controversial.
ERAs play a major role in the management of pulmonary vascular disease. Other drugs, still under study, could prove useful in the treatment of infiltrating pneumonias.Le texte complet de cet article est disponible en PDF.
Keywords : Pulmonary arterial hypertension, Endothelin-receptor antagonists, Bosentan, Ambrisentan, Sitaxentan, Pulmonary fibrosis
| This article is the English translation of the following manuscript, which was originally published in French in Revue des Maladies Respiratoires. Please cite this article as : “Boniface S, Reynaud-Gaubert M. Les antagonistes des récepteurs de l’endothéline : leur place dans les maladies pulmonaires. Rev Mal Respir 2010;27:103–5”.