Skip to main content
SpringerLink
Log in

Pirfenidone in idiopathic pulmonary fibrosis: a guide to its use in the EU

  • Adis Drug Q&A
  • Published:
Drugs & Therapy Perspectives Aims and scope Submit manuscript

Abstract

Pirfenidone (Esbriet®), a synthetic pyridine compound with antifibrotic, anti-inflammatory and antioxidant properties that inhibit the progression of tissue fibrosis, is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). In clinical trials in patients with mild to moderate IPF, oral pirfenidone generally provided a reduced rate of decline in forced vital capacity, while prolonging progression-free survival and reducing the risk of IPF-related and all-cause mortality. Pirfenidone has a manageable tolerability profile; however, gastrointestinal and skin-related events are common. Data from longer-term and real-world studies of pirfenidone use in patients with IPF are consistent with those in clinical trials.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19.

    Article  PubMed  Google Scholar 

  2. Cottin V, Maher T. Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis. Eur Respir Rev. 2015;24(135):58–64.

    Article  PubMed  Google Scholar 

  3. Sandbo N. Mechanisms of fibrosis in IPF. In: Meyer KC, Nathan SD, editors. Idiopathic pulmonary fibrosis: a comprehensive clinical guide: Humana Press; 2014. p. 161–205.

  4. Kim ES, Keating GM. Pirfenidone: a review of its use in idiopathic pulmonary fibrosis. Drugs. 2015;75(2):219–30.

    Article  CAS  PubMed  Google Scholar 

  5. Esbriet (pirfenidone) 267 mg hard capsules: summary of product characteristics. London: European Medicines Agency; 2016.

  6. Oku H, Shimizu T, Kawabata T, et al. Antifibrotic action of pirfenidone and prednisolone: Different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. Eur J Pharmacol. 2008;590(1–3):400–8.

    Article  CAS  PubMed  Google Scholar 

  7. Esbriet (pirfenidone) capsules: US prescribing information. South San Francisco: Genetech USA, Inc.; 2016.

  8. King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92.

    Article  PubMed  Google Scholar 

  9. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9.

    Article  CAS  PubMed  Google Scholar 

  10. Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171(9):1040–7.

    Article  PubMed  Google Scholar 

  11. Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35(4):821–9.

    Article  CAS  PubMed  Google Scholar 

  12. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47(1):243–53.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Nathan SD, Albera C, Bradford WZ, et al. Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax. 2016;71(5):429–35.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Lancaster L, Albera C, Bradford WZ, et al. Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials. BMJ Open Respir Res. 2016;3(1):e000105.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Valeyre D, Albera C, Bradford WZ, et al. Comprehensive assessment of the long-term safety of pirfenidone in patients with idiopathic pulmonary fibrosis. Respirology. 2014;19(5):740–7.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Costabel U, Albera C, Bradford WZ, et al. Analysis of lung function and survival in RECAP: an open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(3):198–205.

    PubMed  Google Scholar 

  17. Ravaglia C, Gurioli C, Romagnoli M, et al. Pirfenidone treatment in idiopathic pulmonary fibrosis: an Italian case series [abstract no. P3370]. Eur Respir J. 2013;42(Suppl. 57):688s.

    Google Scholar 

  18. Bonella F, Wessendorf TE, Costabel U. Clinical experience with pirfenidone for the treatment of idiopathic pulmonary fibrosis [article in German]. Dtsch Med Wochenschr. 2013;138(11):518–23.

    Article  CAS  PubMed  Google Scholar 

  19. Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases. Respiration. 2014;88:199–207.

    Article  CAS  PubMed  Google Scholar 

  20. Lützkendorf L, Vogler M, Hoffken G, et al. Real-life experience with pirfenidon in idiopathic pulmonary fibrosis: does early treatment start predict better therapy adherence? [abstract no. PA3043]. Eur Respir J. 2015;46(Suppl 59).

  21. Wijsenbeek M, Van Beek FT, Geel AL, et al. Pirfenidone in daily clinical use in patients with idiopathic pulmonary fibrosis in the Netherlands [abstract no. A4340]. Am J Respir Crit Care Med. 2013;187(abstract issue).

  22. Chaudhuri N, Duck A, Frank R, et al. Real world experiences: pirfenidone is well tolerated in patients with idiopathic pulmonary fibrosis. Respir Med. 2014;108(1):224–6.

    Article  PubMed  Google Scholar 

  23. Wijsenbeek M, Grutters J, Wuyts W. Early experience of pirfenidone in daily clinical practice in Belgium and the Netherlands: a retrospective cohort analysis. Adv Ther. 2015;32(7):691–704.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Loeh B, Drakopanagiotakis F, Bandelli GP, et al. Intraindividual response to treatment with pirfenidone in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 20151;191(1):110–3.

  25. Harari S, Caminati A, Albera C, et al. Efficacy of pirfenidone for idiopathic pulmonary fibrosis: an Italian real life study. Respir Med. 2015;109(7):904–13.

    Article  CAS  PubMed  Google Scholar 

  26. Nieto A, Valenzuela C, Rivera I, et al. Pirfenidone in idiopathic pulmonary fibrosis (ipf): Spanish experience with the European named patient program (npp) and Spanish patient progam (spp) [abstract no. A1418]. Am J Respir Crit Care Med. 2014;189(abstract issue).

  27. Koschel D, Cottin V, Sköld M, et al. Pirfenidone post-authorization safety registry (PASSPORT)—interim analysis of IPF treatment [abstract no. 850348]. Eur Respir J. 2014;44(Suppl 58).

Download references

Acknowledgments

The review was updated from Drugs 2015;75(2):219–30 [4], and was reviewed by: T.E. Wessendorf, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, Germany. During the peer review process, the manufacturer of pirfenidone was also offered an opportunity to review this article. Changes resulting from comments received were made on the basis of scientific and editorial merit.

Author information

Authors and Affiliations

  1. Springer, Private Bag 65901, Mairangi Bay, 0754, Auckland, New Zealand

    Sarah L. Greig, Katherine A. Lyseng-Williamson, Esther S. Kim & Gillian M. Keating

Authors
  1. Sarah L. Greig
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Katherine A. Lyseng-Williamson
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Esther S. Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gillian M. Keating
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Katherine A. Lyseng-Williamson.

Ethics declarations

Funding

The preparation of this review was not supported by any external funding.

Conflict of interest

S.L. Greig, K.A. Lyseng-Williamson, E.S. Kim and G.M. Keating are salaried employees of Adis/Springer, are responsible for the article content and declare no relevant conflicts of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Greig, S.L., Lyseng-Williamson, K.A., Kim, E.S. et al. Pirfenidone in idiopathic pulmonary fibrosis: a guide to its use in the EU. Drugs Ther Perspect 32, 323–329 (2016). https://doi.org/10.1007/s40267-016-0321-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40267-016-0321-6

Keywords

Search

Navigation