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Abstract
Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic lung disease with a poor prognosis. With advances in understanding of its pathogenesis, antifibrotic agents were proven to be effective. Pirfenidone slows the progression of IPF and fortunately it has resulted in survival benefit.
Areas covered: Randomized clinical trials of pirfenidone showed a significant reduction in the decline of lung function. In 2014 May, the pooled analysis of the results of the Assessment of Pirfenidone to Confirm Efficacy and Safety in IPF trial combined with CAPACITY trials revealed a mortality benefit, which was the first in the history of IPF.
Expert opinion: Pirfenidone is slow acting and is not approved for severe patients except in Japan. The prevalence of its use and compliance are current issues. An important task after marketing is to identify appropriate candidates for its use and the correct time of initiation, in addition to establishing criteria to evaluate its therapeutic benefits. Furthermore, it is desirable to widen the approval of this novel drug for other fibrotic diseases. We need to investigate other unknown therapeutic benefits associated with prognosis other than inhibition of declines in vital capacity.
Declaration of interest
A Azuma is an advisory board member for Boehringer Ingelheim, Shinogi, Takeda Pharmaceuticals and GlaxoSmithKline. He is also a steering committee member for clinical trials with Boehringer Ingelheim, InterMune, AFT Pharmaceuticals and LTT Bio-Pharma. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.