Macitentan reduces progression of TGF-β1-induced pulmonary fibrosis and pulmonary hypertension

Pierre-Simon Bellaye; Toyoshi Yanagihara; Elise Granton; Seidai Sato; Chiko Shimbori; Chandak Upagupta; Jewel Imani; Nathan Hambly; Kjetil Ask; Jack Gauldie; Marc Iglarz; Martin Kolb

doi:10.1183/13993003.01857-2017

Extract

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with unknown cause. Two drugs, nintedanib and pirfenidone, have been shown to slow, but not stop disease progression. Pulmonary hypertension (PH) is a frequent complication in IPF patients associated with poor prognosis. Macitentan is a dual endothelin receptor antagonist which is approved for PAH treatment. We hypothesised that the treatment of AdTGF-β1-induced pulmonary fibrosis animals with macitentan will result in improvement of PH due to chronic lung disease and limitation of fibrosis progression.

Footnotes

This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

Conflict of interest: Dr. Bellaye has nothing to disclose.

Conflict of interest: Dr. Yanagihara has nothing to disclose.

Conflict of interest: Dr. Granton has nothing to disclose.

Conflict of interest: Dr. Sato has nothing to disclose.

Conflict of interest: Dr. Shimbori has nothing to disclose.

Conflict of interest: Dr. Upagupta has nothing to disclose.

Conflict of interest: Dr. Imani has nothing to disclose.

Conflict of interest: Dr. Hambly has nothing to disclose.

Conflict of interest: Dr. Ask has nothing to disclose.

Conflict of interest: Dr. Gauldie has nothing to disclose.

Conflict of interest: Dr. Iglarz reports other from Actelion Pharmaceuticals Ltd., outside the submitted work; .

Conflict of interest: Dr. Kolb reports grants and personal fees from Roche, grants and personal fees from Boehringer Ingelheim, grants and personal fees from GSK, grants and personal fees from Gilead, grants from Actelion, grants from Respivert, personal fees from Astra Zeneca, grants and personal fees from Prometic, personal fees from Genoa, grants and personal fees from Alkermes, grants from Synairgen, outside the submitted work; .

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