Summary Introduction 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. 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. Conclusion 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.