Bosentan for the treatment of idiopathic pulmonary fibrosis

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a rare disease with limited survival in which anarchic fibrosis dominates pathogenesis and immunosuppressive medication is of limited benefit in prolonging survival. Endothelin is able to potentiate TGF-β activity in IPF and its blockade with bosentan, a dual endothelin receptor antagonist could represent a novel therapeutic approach. Objective: To appraise the efficacy and safety data on bosentan for IPF therapy as reported by the BUILD-1 study (Bosentan Use in Interstitial Lung Disease). Methods: This was a randomized, placebo-controlled study performed in subjects with IPF in which bosentan given for 1 year was shown to delay disease progression in patients with biopsy-proven IPF. Results/conclusion: These clinical efficacy data are promising but should be supported by further long-term efficacy data.

Keywords::

• bosentan
• endothelin antagonist
• idiopathic pulmonary fibrosis
• TGF-β