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Pirfenidone: is it tolerable?
  1. Abigail Lara and
  2. Marvin Schwarz
  1. Division of Pulmonary Sciences & Critical Care Medicine, University of Colorado Denver, Aurora, Colorado, USA
  1. Correspondence to Dr Marvin Schwarz;{at}

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Idiopathic pulmonary fibrosis (IPF) remains the commonest and most lethal of all the fibrotic lung diseases. Until recently, clinical studies identifying effective treatments have largely been lacking. Two agents, pirfendione and nintedanib have recently demonstrated clinical efficacy in slowing the rate of physiological decline in patients and improve progression-free survival after 52 weeks of treatment in those who were rigorously diagnosed with IPF.1–3 Pirfendione, an oral pyridine compound, exerts it's effect by targeting multiple pathways that have been implicated in the pathophysiology of IPF.4 The clinical efficacy of pirfendione was evaluated in several phase 3 randomised control trials, which led to the US Food and Drug Administration (FDA) approval for use and has demonstrated a clear improvement in physiological metrics of disease as well as clinically relevant outcomes including reduction in mortality as well as decrease in IPF associated exacerbations and death. Though pirfendione has clearly been proven as a treatment for IPF, the long-term side effects …

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