Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume
Martin Kolb,Luca Richeldi,Jürgen Behr,Toby M. Maher,W. Tang,Susanne Stowasser,Christoph Hallmann,Roland M. du Bois +7 more
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TLDR
Patients with IPF and preserved lung volume (FVC >90% predicted) have the same rate of FVC decline and receive the same benefit from nintedanib as patients with more impaired lung volume.Abstract:
Rationale There is no consensus as to when treatment for idiopathic pulmonary fibrosis (IPF) should be initiated. Some physicians prefer not to treat patients with preserved lung volume.
Objective To investigate whether patients with IPF and preserved lung volume receive the same benefit from nintedanib as patients with more impaired lung volume.
Methods Post hoc subgroup analyses of pooled data from the two replicate phase III INPULSIS trials by baseline FVC % predicted (≤90%, >90%).
Results At baseline, 274 patients had FVC >90% predicted and 787 patients had FVC ≤90% predicted. In patients treated with placebo, the adjusted annual rate of decline in FVC was consistent between patients with FVC >90% predicted and FVC ≤90% predicted (−224.6 mL/year and −223.6 mL/year, respectively). There was no statistically significant difference between these subgroups in the effect of nintedanib on annual rate of decline in FVC, change from baseline in St George's Respiratory Questionnaire total score or time to first acute exacerbation. In patients with baseline FVC >90% predicted and ≤90% predicted, respectively, the adjusted annual rate of decline in FVC with nintedanib was −91.5 mL/year (difference vs placebo: 133.1 mL/year (95% CI 68.0 to 198.2)) and −121.5 mL/year (difference vs placebo: 102.1 mL/year (95% CI 61.9 to 142.3)). Adverse events associated with nintedanib were similar in both subgroups.
Conclusions Patients with IPF and preserved lung volume (FVC >90% predicted) have the same rate of FVC decline and receive the same benefit from nintedanib as patients with more impaired lung volume.
Trial registration number NCT01335464 and [NCT01335477][1].
[1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01335477&atom=%2Fthoraxjnl%2Fearly%2F2016%2F09%2F26%2Fthoraxjnl-2016-208710.atomread more
Citations
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Journal ArticleDOI
Idiopathic Pulmonary Fibrosis.
TL;DR: Idiopathic Pulmonary Fibrosis IdiopATHic pulmonary fibrosis appears to be increasing in incidence, and it requires early recognition and intervention with supportive care and pharmacologic agents to forestall its progression.
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Idiopathic pulmonary fibrosis: pathogenesis and management.
Giacomo Sgalla,Bruno Iovene,Mariarosaria Calvello,Margherita Ori,Francesco Varone,Luca Richeldi +5 more
TL;DR: Building on the advances achieved in the understanding of IPF pathobiology, the further investigation of the role of gene variants, epigenetic alterations and other molecular biomarkers reflecting disease activity and behaviour will hopefully enable earlier and more confident diagnosis, improve disease phenotyping and support the development of novel agents for personalized treatment of IPf.
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The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis
Andreas Guenther,Ekaterina Krauss,Silke Tello,Jasmin Wagner,Bettina Paul,Stefan Kuhn,Olga Maurer,Sabine Heinemann,Ulrich Costabel,María Asunción Nieto Barbero,Veronika Müller,Philippe Bonniaud,Carlo Vancheri,Athol U. Wells,Martina Vasakova,Alberto Pesci,Matteo Sofia,Walter Klepetko,Werner Seeger,Fotios Drakopanagiotakis,Bruno Crestani +20 more
TL;DR: The data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time.
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Idiopathic Pulmonary Fibrosis (IPF): An Overview
TL;DR: Key efforts are directed at identifying key biomarkers that may direct more customized patient-centred healthcare to improve outcomes for patients with IPF, with several other agents in early clinical trials.
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Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat.
TL;DR: There remains a need to educate pulmonologists that IPF is a progressive, irreversible and fatal disease and that prompt treatment is critical to preserving patients’ lung function and improving outcomes.
References
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An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management
Ganesh Raghu,Harold R. Collard,Jim J. Egan,Fernando J. Martinez,Juergen Behr,Kevin K. Brown,Thomas V. Colby,Jean-François Cordier,Kevin R. Flaherty,Joseph A. Lasky,David A. Lynch,Jay H. Ryu,Jeffrey J. Swigris,Athol U. Wells,Julio Ancochea,Demosthenes Bouros,Carlos Roberto Ribeiro de Carvalho,Ulrich Costabel,Masahito Ebina,David M. Hansell,Takeshi Johkoh,Dong Soon Kim,Talmadge E. King,Yasuhiro Kondoh,Jeffrey L. Myers,Nestor L. Müller,Andrew G. Nicholson,Luca Richeldi,Moisés Selman,Rosalind F. Dudden,Barbara S. Griss,Shandra Protzko,Holger J. Schünemann +32 more
TL;DR: This document represents the current state of knowledge regarding idiopathic pulmonary fibrosis, and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course.
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Lung volumes and forced ventilatory flows
TL;DR: Assessing the total lung capacity is indispensable in establishing a restrictive ventilatory defect or in diagnosing abnormal lung distensibility, as may occur in patients …
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Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis
Luca Richeldi,Roland M. du Bois,Ganesh Raghu,Arata Azuma,Kevin K. Brown,Ulrich Costabel,Vincent Cottin,Kevin R. Flaherty,David M. Hansell,Yoshikazu Inoue,Dong Soon Kim,Martin Kolb,Andrew G. Nicholson,Paul W. Noble,Moisés Selman,Hiroyuki Taniguchi,Michèle Brun,Florence Le Maulf,Mannaig Girard,Susanne Stowasser,Rozsa Schlenker-Herceg,Bernd Disse,Harold R. Collard +22 more
TL;DR: In patients with idiopathic pulmonary fibrosis, nintedanib reduced the decline in FVC, which is consistent with a slowing of disease progression; nintinganib was frequently associated with diarrhea, which led to discontinuation of the study medication in less than 5% of patients.
Journal ArticleDOI
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis
Talmadge E. King,Williamson Z. Bradford,Socorro Castro-Bernardini,Elizabeth A. Fagan,Ian Glaspole,Marilyn K. Glassberg,Eduard Gorina,Peter Hopkins,David Kardatzke,Lisa Lancaster,David J. Lederer,Steven D. Nathan,Carlos Alberto de Castro Pereira,Steven A. Sahn,Robert Sussman,Jeffrey J. Swigris,Paul W. Noble +16 more
TL;DR: Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with idiopathic pulmonary fibrosis.
Idiopathic Pulmonary Fibrosis
TL;DR: The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough; many affected individuals also experience a loss of appetite and gradual weight loss.
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