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Idiopathic Pulmonary Fibrosis pp 147–159Cite as

Pharmacotherapy of IPF Using Antifibrotic Compounds

Can Antifibrotic Agents Rescue Patients with IPF?

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Abstract

Pirfenidone is an antifibrotic agent that has multiple functions, and three out of four phase III clinical trials proved its efficacy to suppress disease progression in patients with mild to moderate idiopathic pulmonary fibrosis (IPF). In addition, pooled data have shown its efficacy to improve survival of patients with IPF. Nintedanib is another drug whose efficacy in the treatment of IPF has been confirmed by clinical trials, and both these drugs have recently been approved by the FDA. However, the efficacy of both drugs in severe IPF and the optimal length of therapy remain unknown. Based on the balance of clinical efficacy, side effects, disease severity itself, and cost-effectiveness, neither of these drugs is strongly recommended in the international guidelines. Furthermore, inhaling NAC is a cost-effective therapy, and well-conducted RCT should be considered to assess its effectiveness in treating IPF. Because a substantial percentage of patients with IPF die from acute exacerbation or lung cancer, medical treatment for IPF should focus not only on slowing the disease progression, but also on reducing the risk of acute exacerbation and lung cancer. Further investigation regarding the efficacy of combination therapies is necessary.

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Author information

Authors and Affiliations

  1. Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University Hospital, 54 kawaharacho Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan

    Tomohiro Handa

  2. Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo, Tokyo, 113-8603, Japan

    Arata Azuma

Authors
  1. Tomohiro Handa
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  2. Arata Azuma
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Correspondence to Arata Azuma .

Editor information

Editors and Affiliations

  1. Department of Respiratory Medicine, Tokyo Medical University, Ibaraki Medical Center , Ibaraki, Japan

    Hiroyuki Nakamura

  2. Department of Respiratory Medicine , Tokyo Medical University, Ibaraki Medical Center, Ibaraki, Japan

    Kazutetsu Aoshiba

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Handa, T., Azuma, A. (2016). Pharmacotherapy of IPF Using Antifibrotic Compounds. In: Nakamura, H., Aoshiba, K. (eds) Idiopathic Pulmonary Fibrosis. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55582-7_9

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  • DOI: https://doi.org/10.1007/978-4-431-55582-7_9

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-55581-0

  • Online ISBN: 978-4-431-55582-7

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