Bosentan is an endothelin receptor antagonist, displacing endothelin-1. This removes the effect of endothelin-1. As a result, blood vessels dilate and (pulmonary) blood pressure decreases. Bosentan is an orphan drug used to treat rare pulmonary arterial hypertension (pulmonary blood pressure), it has recently been used quite successfully for scleroderma (autoimmune connective tissue disease).
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