Targeting the endothelin pathway in the idiopathic pulmonary fibrosis: the role of bosentan.

摘要

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a rapidly lethal disease characterized by anarchic, progressive fibrosis. Pulmonary fibrosis is the result of interactions between many effector cells and cytokines and better understanding of this can help with identification of novel therapeutic targets. OBJECTIVE: To evaluate the role of the endothelin-1 (ET-1) pathway in IPF pathogenesis and the effects of therapeutic targeting with bosentan, an ET-1 antagonist. METHODS: Data on ET-1's pathogenic involvement in IPF and the preclinical and clinical data on bosentan in this context are discussed and analyzed. A parallel overview of existing and upcoming therapies for IPF is presented. CONCLUSIONS: Bosentan is a promising antifibrotic therapy for IPF and clinical data on its long-term efficacy support its use.