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Observational Study
. 2018 Nov 23;18(1):177.
doi: 10.1186/s12890-018-0736-z.

Pirfenidone improves survival in IPF: results from a real-life study

George A Margaritopoulos  1   2 , Athina Trachalaki  1 , Athol U Wells  2 , Eirini Vasarmidi  1 , Eleni Bibaki  1 , George Papastratigakis  1 , Stathis Detorakis  3 , Nikos Tzanakis  1 , Katerina M Antoniou  4
Affiliations
Observational Study

Pirfenidone improves survival in IPF: results from a real-life study

George A Margaritopoulos et al. BMC Pulm Med. .

Abstract

Background: Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone's effect on mortality and adverse events profile outside the restrictions of a clinical trial.

Methods: This is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort).

Results: We observed a high 3-years survival rate of 73% without excluding patients who discontinued treatment for different reasons. The survival was compared to the survival of an IPF cohort from a tertiary referral center (RBH cohort). After exclusion of severe cases (DLco< 30%), in unadjusted analysis, the survival in the UHH cohort was better than in the RBH cohort (HR:0.32, 95% CI: 0.19-0.53, p < 0.0001). After adjustment for age, gender and FVC, the survival remained higher in the UHH cohort (HR:0.28, 95% CI: 0.16-0.48, p < 0.0001). We observed a similar safety profile compared to previously published data and a lower rate of drug discontinuation due to photosensitivity reactions.

Conclusion: Pirfenidone provides a survival benefit in a real-life IPF cohort compared to previously used medications. Counselling patients and proactively managing possible adverse effects can reduce the necessity to discontinue pirfenidone.

Keywords: IPF; Pirfenidone; Real-life; Survival.

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Conflict of interest statement

Ethics approval and consent to participate

Informed consent was obtained from all patients included in the UHH cohort. The study was approved by the Ethics Committees of the University Hospital of Herakleion (IRB number: 17030). Due to the retrospective nature of the analysis of the RBH cohort13, ethics committee approval was not required.

Consent for publication

Not applicable

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Kaplan Meier survival curve for the whole UHH cohort
Fig. 2
Fig. 2
Kaplan Meier survival curve after adjustment for FVC, age and gender
See this image and copyright information in PMC

References

    1. Margaritopoulos G. A., Romagnoli M., Poletti V., Siafakas N. M., Wells A. U., Antoniou K. M. Recent advances in the pathogenesis and clinical evaluation of pulmonary fibrosis. European Respiratory Review. 2012;21(123):48–56. doi: 10.1183/09059180.00007611. - DOI - PMC - PubMed
    1. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2016. CA Cancer J Clin. 2016;66(1):7–30. doi: 10.3322/caac.21332. - DOI - PubMed
    1. Hutchinson J, Fogarty A, Hubbard R, McKeever T. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015;46(3):795–806. doi: 10.1183/09031936.00185114. - DOI - PubMed
    1. Margaritopoulos GA, Vasarmidi E, Antoniou KM. Pirfenidone in the treatment of idiopathic pulmonary fibrosis: an evidence-based review of its place in therapy. Core Evidence. 2016;11:11–22. doi: 10.2147/CE.S76549. - DOI - PMC - PubMed
    1. Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J, Brozek JL, Collard HR, Cunningham W, Homma S, 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. doi: 10.1164/rccm.201506-1063ST. - DOI - PubMed

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