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The Effectiveness, Safety, and Tolerability of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Retrospective Study

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Abstract

Introduction

In this study we aimed to investigate the effectiveness and safety profile of pirfenidone for the treatment of idiopathic pulmonary fibrosis (IPF) in a real-life setting.

Methods

Clinical records of patients diagnosed with mild-to-moderate IPF and receiving pirfenidone treatment across three centers in Turkey between January and September 2017 were retrospectively collected. Pulmonary function measurements, including percentage of forced vital capacity (FVC%) and percentage of diffusion capacity (DLCO%) were analyzed in patients who received pirfenidone treatment for at least 6 months. Decline in lung function, defined as an absolute decline of at least 10% in FVC from baseline, or death at 6 months was also analyzed. Safety data were included for all follow-up visits.

Results

In the pooled cohort (n = 60), patients were mostly men (73.4%) and current or former smokers (61.7%). Average baseline FVC% and DLCO% were 68.4% and 48.7%, respectively. Forty-seven patients (78.3%) had a high-resolution computed tomography scan with a definite interstitial pneumonia (UIP) pattern, and 18 patients (30%) had a surgically proven UIP pattern. Forty-six (76.7%) patients with IPF remained stable and 14 (23.3%) patients had progressed according to decline in FVC of at least 10% during the therapy course. After 6 months of therapy, cough decreased in 58.3% of patients. At least one side effect due to therapy was encountered in 33 (55.0%) IPF patients. Dyspepsia (36.4%), nausea (27.3%), and rash/photosensitivity (24.2%) were the most frequent side effects in our cohort. Sixteen patients (26.7%) needed dose adjustment, one patient (1.7%) discontinued therapy, and one patient (1.7%) died in the study period.

Conclusions

This study shows that pirfenidone seems to be an effective treatment for IPF and also had tolerable and relatively acceptable side effects.

Funding

Roche.

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References

  1. Raghu G. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Raghu G. 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:e3–e19.

    Article  PubMed  Google Scholar 

  3. 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;171:1040–7.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  6. 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;370:2083–92.

    Article  CAS  PubMed  Google Scholar 

  7. 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:e000105.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Costabel U, Albera C, Lancaster LH, et al. An open-label study of the long term safety of pirfenidone in patients with idiopathic pulmonary fibrosis (RECAP). Respiration. 2017;94:408–15.

    Article  CAS  PubMed  Google Scholar 

  9. Nathan SD, Albera C, Bradford WZ. 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;71:429–35.

    Article  PubMed  Google Scholar 

  10. Ley B, Swigris J, Day BM. Pirfenidone reduces respiratory-related hospitalizations in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2017;196(6):756–61.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Van Manen MJG, Birring SS, Vancheri C, et al. Effect of pirfenidone on cough in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2017;50(4):1701157.

  12. Oltmanns U, Kahn N, Palmowski K, Trager A, Wenz H, Heussel CP. 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 

  13. Lancaster L, de Andrade JA, Zibrak JD, Padilla ML, Albera C, Nathan SD. Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis. Eur Respir Rev. 2017;26(146):170057.

    Article  PubMed  Google Scholar 

  14. Tzouvelekis A, Karampitsakos T, Ntolios P, Tzilas V, Bouros E, Markozannes E. Longitudinal “real-world” outcomes of pirfenidone in idiopathic pulmonary fibrosis in Greece. Front Med (Lausanne). 2017;29(4):213.

  15. Salih GN, Shaker SB, Madsen HD, Bendstrup E. Pirfenidone treatment in idiopathic pulmonary fibrosis: nationwide Danish results. Eur Clin Respir J. 2016;9(3):32608.

    Article  Google Scholar 

  16. Skold CM, Janson C, Elf AK, Fiaschi M, Wiklund K, Persson HL. A retrospective chart review of pirfenidone-treated patients in Sweden: the REPRIS study. Eur Clin Respir J. 2016;18(3):32035.

    Article  Google Scholar 

  17. Chaudhuri N, Duck A, Frank R, Holme J, Leonard C. Real world experiences: pirfenidone is well tolerated in patients with idiopathic pulmonary fibrosis. Respir Med. 2014;108:224–6.

  18. Cottin V, Koschel D, Günther A. Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study. ERJ Open Res. 2018:19:00084-2018. https://doi.org/10.1183/23120541.00084-2018.

    Article  Google Scholar 

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Acknowledgements

Funding

The study and the article processing charges were funded by an unrestricted grant from Roche. The funding source had no input in the design, data collection and analysis and writing of the manuscript. All authors had full access to all of the data in this study and take complete responsibility for the integrity of the data and accuracy of the data analysis

Authorship

All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article, take responsibility for the integrity of the work as a whole, and have given their approval for this version to be published.

Disclosures

İsmail Hanta, Aykut Cilli and Can Sevinc have nothing to disclose.

Compliance with Ethics Guidelines

This multicenter, retrospective research was conducted on the archive records of three chest diseases departments. For this kind of research, to obtain ethical committee approval was not an obligation, legally in Turkey and institutionally in Çukurova University.

Data Availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

Author information

Authors and Affiliations

  1. Department of Chest Diseases, Çukurova University, Adana, Turkey

    Ismail Hanta

  2. Department of Chest Diseases, Akdeniz University, Antalya, Turkey

    Aykut Cilli

  3. Department of Chest Diseases, Dokuz Eylül University, İzmir, Turkey

    Can Sevinc

Authors
  1. Ismail Hanta
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  2. Aykut Cilli
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  3. Can Sevinc
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Corresponding author

Correspondence to Ismail Hanta.

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Hanta, I., Cilli, A. & Sevinc, C. The Effectiveness, Safety, and Tolerability of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Retrospective Study. Adv Ther 36, 1126–1131 (2019). https://doi.org/10.1007/s12325-019-00928-3

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  • DOI: https://doi.org/10.1007/s12325-019-00928-3

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