Literature DB >> 31153107

Pirfenidone in patients with idiopathic pulmonary fibrosis and more advanced lung function impairment.

Steven D Nathan1, Ulrich Costabel2, Carlo Albera3, Jürgen Behr4, Wim A Wuyts5, Klaus-Uwe Kirchgaessler6, John L Stauffer7, Elizabeth Morgenthien8, Willis Chou9, Susan L Limb10, Paul W Noble11.   

Abstract

BACKGROUND: Patients with idiopathic pulmonary fibrosis (IPF) demonstrate a range of lung function impairment. However, the efficacy of antifibrotics compared with placebo has not been assessed in patients with more advanced disease. This post-hoc analysis investigated the efficacy and safety of pirfenidone versus placebo in patients with IPF and more advanced lung function impairment, defined as percent predicted forced vital capacity (%FVC) < 50% and/or percent predicted carbon monoxide diffusing capacity <35%.
METHODS: Patients randomised to pirfenidone 2,403 mg/day or placebo in the ASCEND (NCT01366209) and CAPACITY (NCT00287716; NCT00287729) trials with more advanced baseline lung function impairment (pirfenidone, n = 90; placebo, n = 80) were included. Mortality, lung function, hospitalisation, exercise capacity and dyspnoea were investigated over 52 weeks.
RESULTS: At Week 52 versus placebo, pirfenidone was associated with significantly lower risks of all-cause mortality (hazard ratio [HR] 0.28; 95% confidence interval [CI] 0.09-0.86; p=0.0180), ≥10% absolute %FVC decline or all-cause mortality (HR 0.40; 95% CI 0.23-0.69; p=0.0006) and ≥10% absolute %FVC decline or respiratory-related hospitalisation or all-cause mortality (HR 0.46; 95% CI 0.28-0.76; p=0.0018). At Week 52, median treatment differences favouring pirfenidone were 36.7 m for 6-min walk distance and -8.0 points for the University of California-San Diego Shortness of Breath Questionnaire total score. Treatment-emergent adverse events (TEAEs) led to discontinuation in 14.4% and 21.3% of patients with pirfenidone and placebo, respectively.
CONCLUSION: Pirfenidone demonstrated clinically relevant benefits across multiple domains in patients with IPF and more advanced disease without an increased risk of discontinuation due to TEAEs. CLINICAL TRIALS REGISTRATION: clinicaltrials. gov (ASCEND: NCT01366209; CAPACITY: NCT00287716; NCT00287729).
Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.

Entities:  

Keywords:  Dyspnoea; Idiopathic pulmonary fibrosis; Mortality; Pirfenidone

Year:  2019        PMID: 31153107     DOI: 10.1016/j.rmed.2019.04.016

Source DB:  PubMed          Journal:  Respir Med        ISSN: 0954-6111            Impact factor:   3.415


  20 in total

1.  Effect of Co-trimoxazole (Trimethoprim-Sulfamethoxazole) vs Placebo on Death, Lung Transplant, or Hospital Admission in Patients With Moderate and Severe Idiopathic Pulmonary Fibrosis: The EME-TIPAC Randomized Clinical Trial.

Authors:  Andrew M Wilson; Allan B Clark; Tony Cahn; Edwin R Chilvers; William Fraser; Matthew Hammond; David M Livermore; Toby M Maher; Helen Parfrey; Ann Marie Swart; Susan Stirling; David R Thickett; Moira Whyte
Journal:  JAMA       Date:  2020-12-08       Impact factor: 56.272

Review 2.  Pulmonary rehabilitation for interstitial lung disease.

Authors:  Leona Dowman; Catherine J Hill; Anthony May; Anne E Holland
Journal:  Cochrane Database Syst Rev       Date:  2021-02-01

3.  Long-Term Follow-Up of Patients With Idiopathic Pulmonary Fibrosis Treated With Pirfenidone or Nintedanib: A Real-Life Comparison Study.

Authors:  Paolo Cameli; Rosa Metella Refini; Laura Bergantini; Miriana d'Alessandro; Valerio Alonzi; Carlo Magnoni; Paola Rottoli; Piersante Sestini; Elena Bargagli
Journal:  Front Mol Biosci       Date:  2020-09-04

Review 4.  Nutrition in Patients with Idiopathic Pulmonary Fibrosis: Critical Issues Analysis and Future Research Directions.

Authors:  Paola Faverio; Marialuisa Bocchino; Antonella Caminati; Alessia Fumagalli; Monica Gasbarra; Paola Iovino; Alessandra Petruzzi; Luca Scalfi; Alfredo Sebastiani; Anna Agnese Stanziola; Alessandro Sanduzzi
Journal:  Nutrients       Date:  2020-04-17       Impact factor: 5.717

5.  Changes in management of idiopathic pulmonary fibrosis: impact on disease severity and mortality.

Authors:  Charlotte Hyldgaard; Janne Møller; Elisabeth Bendstrup
Journal:  Eur Clin Respir J       Date:  2020-08-12

6.  Health-related quality of life and symptoms in patients with IPF treated with nintedanib: analyses of patient-reported outcomes from the INPULSIS® trials.

Authors:  Michael Kreuter; Wim A Wuyts; Marlies Wijsenbeek; Sabrina Bajwah; Toby M Maher; Susanne Stowasser; Natalia Male; Wibke Stansen; Nils Schoof; Leticia Orsatti; Jeffrey Swigris
Journal:  Respir Res       Date:  2020-01-30

7.  Real-world experiences: Efficacy and tolerability of pirfenidone in clinical practice.

Authors:  Chuling Fang; Hui Huang; Jian Guo; Martin Ferianc; Zuojun Xu
Journal:  PLoS One       Date:  2020-01-30       Impact factor: 3.240

8.  Nintedanib and Sildenafil in Patients with Idiopathic Pulmonary Fibrosis. Echoes of the Past, Lessons for the Future.

Authors:  Steven D Nathan
Journal:  Am J Respir Crit Care Med       Date:  2019-12-15       Impact factor: 21.405

9.  Mesenchymal growth hormone receptor deficiency leads to failure of alveolar progenitor cell function and severe pulmonary fibrosis.

Authors:  Ting Xie; Vrishika Kulur; Ningshan Liu; Nan Deng; Yizhou Wang; Simon Coyle Rowan; Changfu Yao; Guanling Huang; Xue Liu; Forough Taghavifar; Jiurong Liang; Cory Hogaboam; Barry Stripp; Peter Chen; Dianhua Jiang; Paul W Noble
Journal:  Sci Adv       Date:  2021-06-09       Impact factor: 14.136

Review 10.  Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic.

Authors:  Alyson W Wong; Lee Fidler; Veronica Marcoux; Kerri A Johannson; Deborah Assayag; Jolene H Fisher; Nathan Hambly; Martin Kolb; Julie Morisset; Shane Shapera; Christopher J Ryerson
Journal:  Chest       Date:  2020-04-22       Impact factor: 9.410

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