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2015 Guidelines for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing form of interstitial pneumonia, with poor survival rates of nearly 50% at 3 years. There are new joint guidelines for IPF from a conglomerate of international experts including the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and the Latin American Thoracic Association (Citation source

The guidelines make strong or conditional recommendations for or against specific agents. Strong recommendations are made against the use of warfarin; imatinib (a selective tyrosine kinase inhibitor); and ambrisentan (a selective endothelin receptor antagonist). Conditional recommendations are made against sildenafil  (a phosphodiesterase-5 inhibitor); and the dual endothelin receptor antagonists, macitentan and bosentan. Two new agents have been given conditional recommendations: nintedanib, a tyrosine kinase inhibitor; and pirfenidone, an oral antifibrotic drug. Pooled data on nintedanib show no significant effect on mortality or acute exacerbations of IPF, but a reduction in decline of forced vital capacity (FVC). Available trial data for pirfenidone show both a reduction in mortality and a reduced rate of FVC decline.

The author has no conflicts of interest to disclose related to this subject

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