Sabina A Antoniu1. 1. Gr.T.Popa University of Medicine and Pharmacy, Iasi, Romania. sabina.antonela.antoniu@pneum.umfiasi.ro
Abstract
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.
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.
Authors: Priya Kulasekaran; Casey A Scavone; David S Rogers; Douglas A Arenberg; Victor J Thannickal; Jeffrey C Horowitz Journal: Am J Respir Cell Mol Biol Date: 2009-02-02 Impact factor: 6.914