Key result
Bosentan demonstrates efficacy across various forms of pulmonary arterial hypertension and appears promising in idiopathic pulmonary fibrosis without pulmonary hypertension.
Why the study?
Does bosentan improve outcomes in patients with pulmonary hypertension and other pulmonary diseases?
Does bosentan improve outcomes in patients with pulmonary hypertension and other pulmonary diseases?
Bosentan is an effective treatment for various forms of pulmonary hypertension and shows promise in other pulmonary diseases and combination therapies.
Supports bosentan in pulmonary arterial hypertension; extends promise to idiopathic pulmonary fibrosis but leaves open randomized confirmation.
Endothelin-1 is a potent vasoconstrictor and mitogen that is primarily synthesized and released from vascular endothelial cells. Bosentan is a dual endothelin-receptor antagonist that initially received approval for treatment of WHO group I pulmonary arterial hypertension (PAH) for patients in functional classes III and IV. Analysis of a study conducted in functional class II patients (Endothelin Antagonist Trial in Mildly Symptomatic PAH Patients [EARLY] trial) suggest its efficacy for these less symptomatic patients. In addition, bosentan has demonstrated efficacy in patients with congenital heart disease and Eisenmengers syndrome with right to left shunting and in HIV-related PAH. Studies of bosentan in inoperable or residual chronic thromboembolic pulmonary hypertension suggest possible efficacy. Bosentan appears promising in patients with idiopathic pulmonary fibrosis who do not have pulmonary hypertension. Combinations of bosentan with other PAH therapies such as iloprost and sildenafil may have incremental benefit over monotherapy.
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Robert P. Frantz (2008) conducted a review in Pulmonary Hypertension and Other Pulmonary Diseases. Bosentan was evaluated. Bosentan demonstrates efficacy across various forms of pulmonary arterial hypertension and appears promising in idiopathic pulmonary fibrosis without pulmonary hypertension.
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