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

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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.

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Most cited references 24

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Cancer statistics, 2016.

Rebecca L Siegel, Kimberly D Miller, Ahmedin Jemal (2016)

Each year, the American Cancer Society estimates the numbers of new cancer cases and deaths that will occur in the United States in the current year and compiles the most recent data on cancer incidence, mortality, and survival. Incidence data were collected by the National Cancer Institute (Surveillance, Epidemiology, and End Results [SEER] Program), the Centers for Disease Control and Prevention (National Program of Cancer Registries), and the North American Association of Central Cancer Registries. Mortality data were collected by the National Center for Health Statistics. In 2016, 1,685,210 new cancer cases and 595,690 cancer deaths are projected to occur in the United States. Overall cancer incidence trends (13 oldest SEER registries) are stable in women, but declining by 3.1% per year in men (from 2009-2012), much of which is because of recent rapid declines in prostate cancer diagnoses. The cancer death rate has dropped by 23% since 1991, translating to more than 1.7 million deaths averted through 2012. Despite this progress, death rates are increasing for cancers of the liver, pancreas, and uterine corpus, and cancer is now the leading cause of death in 21 states, primarily due to exceptionally large reductions in death from heart disease. Among children and adolescents (aged birth-19 years), brain cancer has surpassed leukemia as the leading cause of cancer death because of the dramatic therapeutic advances against leukemia. Accelerating progress against cancer requires both increased national investment in cancer research and the application of existing cancer control knowledge across all segments of the population.

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An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

Ganesh Raghu, Harold Collard, Jim J. J. Egan … (2011)

American Journal of Respiratory and Critical Care Medicine, 183(6), 788-824

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Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.

Luca Richeldi, Roland M du Bois, Ganesh Raghu … (2014)

Nintedanib (formerly known as BIBF 1120) is an intracellular inhibitor that targets multiple tyrosine kinases. A phase 2 trial suggested that treatment with 150 mg of nintedanib twice daily reduced lung-function decline and acute exacerbations in patients with idiopathic pulmonary fibrosis. We conducted two replicate 52-week, randomized, double-blind, phase 3 trials (INPULSIS-1 and INPULSIS-2) to evaluate the efficacy and safety of 150 mg of nintedanib twice daily as compared with placebo in patients with idiopathic pulmonary fibrosis. The primary end point was the annual rate of decline in forced vital capacity (FVC). Key secondary end points were the time to the first acute exacerbation and the change from baseline in the total score on the St. George's Respiratory Questionnaire, both assessed over a 52-week period. A total of 1066 patients were randomly assigned in a 3:2 ratio to receive nintedanib or placebo. The adjusted annual rate of change in FVC was -114.7 ml with nintedanib versus -239.9 ml with placebo (difference, 125.3 ml; 95% confidence interval [CI], 77.7 to 172.8; P<0.001) in INPULSIS-1 and -113.6 ml with nintedanib versus -207.3 ml with placebo (difference, 93.7 ml; 95% CI, 44.8 to 142.7; P<0.001) in INPULSIS-2. In INPULSIS-1, there was no significant difference between the nintedanib and placebo groups in the time to the first acute exacerbation (hazard ratio with nintedanib, 1.15; 95% CI, 0.54 to 2.42; P=0.67); in INPULSIS-2, there was a significant benefit with nintedanib versus placebo (hazard ratio, 0.38; 95% CI, 0.19 to 0.77; P=0.005). The most frequent adverse event in the nintedanib groups was diarrhea, with rates of 61.5% and 18.6% in the nintedanib and placebo groups, respectively, in INPULSIS-1 and 63.2% and 18.3% in the two groups, respectively, in INPULSIS-2. In patients with idiopathic pulmonary fibrosis, nintedanib reduced the decline in FVC, which is consistent with a slowing of disease progression; nintedanib was frequently associated with diarrhea, which led to discontinuation of the study medication in less than 5% of patients. (Funded by Boehringer Ingelheim; INPULSIS-1 and INPULSIS-2 ClinicalTrials.gov numbers, NCT01335464 and NCT01335477.).

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Author and article information

Contributors

George A. Margaritopoulos: gmargaritop@yahoo.gr

Athina Trachalaki:

ORCID: http://orcid.org/0000-0002-3045-9387

+302810 392433 , atrachalaki@gmail.com

Athol U. Wells: rbhild@rbht.nhs.uk

Eirini Vasarmidi: eirvasar@gmail.com

Eleni Bibaki: bibakieleni@gmail.com

George Papastratigakis: papastratigakisg@gmail.com

Stathis Detorakis: Edetorakis@hotmail.com

Nikos Tzanakis: tzanakis@med.uoc.gr

Katerina M. Antoniou: +302810 392433 , kantoniou@med.uoc.gr

Journal

Journal ID (nlm-ta): BMC Pulm Med

Journal ID (iso-abbrev): BMC Pulm Med

Title: BMC Pulmonary Medicine

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2466

Publication date (Electronic): 23 November 2018

Publication date PMC-release: 23 November 2018

Publication date Collection: 2018

Volume: 18

Electronic Location Identifier: 177

Affiliations

[1 ]GRID grid.412481.a, Respiratory Medicine Department, , University Hospital of Heraklion, ; Crete, Greece

[2 ]GRID grid.439338.6, Interstitial Lung Disease Unit, , Royal Brompton Hospital, ; London, UK

[3 ]GRID grid.412481.a, Radiology Department, , University Hospital of Heraklion, ; Crete, Greece

Author information

Athina Trachalaki http://orcid.org/0000-0002-3045-9387

Article

Publisher ID: 736

DOI: 10.1186/s12890-018-0736-z

PMC ID: 6251092

PubMed ID: 30470213

SO-VID: 1905d322-0c50-400b-8ef9-8947b0eca86f

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 25 June 2018

Date accepted : 7 November 2018

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ScienceOpen disciplines: Respiratory medicine

Keywords: ipf,survival,pirfenidone,real-life

Data availability:

ScienceOpen disciplines: Respiratory medicine

Keywords: ipf, survival, pirfenidone, real-life

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

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Most cited references 24

Cancer statistics, 2016.

An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.

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