[Endothelin receptor antagonists - their role in pulmonary medicine]

There has been an explosion of interest in the biology of endothelin-1 in endothelium disease's physiopathology and in particular pulmonary arterial hypertension (PAH). This interest led up to the development of endothelin-receptor antagonists (ERA) drugs as major therapy for advanced PAH.

Three active ERA drugs (bosentan, sitaxentan, ambrisentan) are nowadays approved for the treatment of PAH. Several randomised clinical trials have demonstrated their efficacy and safety in such domain.

Besides its vasoconstrictive effect, endothelin-1 (ET-1) plays a pivotal role on cell proliferation and apoptosis. ET-1 is a particularly interesting molecule linking idiopathic pulmonary fibrosis or systemic sclerosis, although preliminary results are still insufficient or controversial to conclude.

The role of endothelin-1 in the pathogenesis of fibrosis and angiogenesis needs to be clarified and the interest of ERA in the diseases in which such injuries are preponderant might be demonstrated by further clinical trials.