The Medical Letter on Drugs and Therapeutics
Two New Drugs for Idiopathic Pulmonary Fibrosis
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The FDA has approved 2 oral drugs for treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone (Esbriet) has been available in Europe since 2011 and in Canada since 2012. Nintedanib (Ofev) is available only in the US.

THE DISEASE — IPF is a chronic, progressive disease with a median survival of 3 years that primarily occurs in adults ≥50 years old. It is characterized by abnormal fibroproliferation that leads to scarring and destruction of the lungs. The only treatment shown to prolong survival in patients with advanced IPF is lung transplant; the post-transplant 5-year survival rate is about 44%.1

MECHANISM OF ACTION — The exact mechanism of action of pirfenidone is unknown, but the drug is thought to downregulate profibrotic growth factors, particularly transforming growth factor ... more      

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Title: Two New Drugs for Idiopathic Pulmonary Fibrosis
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