title: Endothelin receptor antagonists -- their role in pulmonary medicine.
abstract: BACKGROUND
  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.
  
  
  BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Cabinet de Pneumologie, 4, avenue de Delphes, 13006 Marseille, France.
authors.forename: S
authors.initials: S
authors.lastname: Boniface
authors.forename: M
authors.initials: M
authors.lastname: Reynaud-Gaubert
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D065128:Endothelin Receptor Antagonists; D016232:Endothelins / Q000037:antagonists & inhibitors; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy; D008954:Models, Biological; D015272:Pulmonary Medicine / Q000379:methods*
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D016232:Endothelins