Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease
Kevin R. Flaherty,Kevin K. Brown,Athol U. Wells,Emmanuelle Clerisme-Beaty,Harold R. Collard,Vincent Cottin,Anand Devaraj,Yoshikazu Inoue,Florence Le Maulf,Luca Richeldi,Hendrik Schmidt,Simon L.F. Walsh,William Mezzanotte,Rozsa Schlenker-Herceg +13 more
TLDR
The main secondary endpoints are the absolute change from baseline in King’s Brief Interstitial Lung Disease Questionnaire total score, time to first acute interstitial lung disease exacerbation or death and time to all-cause mortality over 52 weeks.Abstract:
600 patients aged ≥18 years will be randomised in a 1:1 ratio to nintedanib or placebo. Patients with diagnosis of IPF will be excluded. The study population will be enriched with two-thirds having a usual interstitial pneumonia-like pattern on HRCT. The primary endpoint is the annual rate of decline in forced vital capacity over 52 weeks. The main secondary endpoints are the absolute change from baseline in King’s Brief Interstitial Lung Disease Questionnaire total score, time to first acute interstitial lung disease exacerbation or death and time to all-cause mortality over 52 weeks. Ethics and dissemination The trial is conducted in accordance with the Declaration of Helsinki, the International Conference on Harmonisation Tripartite Guideline for Good Clinical Practice (GCP) and Japanese GCP regulations. Trial registration number NCT02999178.read more
Citations
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Journal ArticleDOI
Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
TL;DR: In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the FVC was significantly lower among patients who received nintedanib than among those who received placebo.
Journal ArticleDOI
Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
Vincent Cottin,Nikhil Hirani,David L. Hotchkin,Anoop M. Nambiar,Takashi Ogura,Maria Otaola,Dirk Skowasch,Jong Sun Park,Hataya K. Poonyagariyagorn,Wim A. Wuyts,Athol U. Wells +10 more
TL;DR: Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype, and identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management.
Journal ArticleDOI
Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial
Athol U. Wells,Kevin R. Flaherty,Kevin K. Brown,Yoshikazu Inoue,Anand Devaraj,Luca Richeldi,Teng Moua,Bruno Crestani,Wim A. Wuyts,Susanne Stowasser,Manuel Quaresma,Rainer Georg Goeldner,Rozsa Schlenker-Herceg,Martin Kolb,S. Abe,Myriam Aburto,O. Acosta,C. Andrews,Danielle Antin-Ozerkis,G. Arce,M. Arias,Sergey Avdeev,A. Barczyk,Rebecca Bascom,E. Bazdyrev,Paul Beirne,E. Belloli,M. A. Bergna,E. Bergot,Nitin Y. Bhatt,S. Blaas,Benjamin Bondue,Francesco Bonella,E. Britt,Ketan P. Buch,J. Burk,H. Cai,A. Cantin,D. M. Castillo Villegas,A. Cazaux,S. Cerri,S. Chaaban,Nazia Chaudhuri,Vincent Cottin,B. Crestani,G. Criner,Caroline Dahlqvist,Sonye K. Danoff,J. Dematte D'Amico,Daniel F. Dilling,P. Elias,Neil Ettinger,J. Falk,E. R. Fernández Pérez,A. Gamez-Dubuis,G. Giessel,A. Gifford,Marilyn K. Glassberg,C. Glazer,J. Golden,L. Gómez Carrera,Julien Guiot,R. Hallowell,H. Hayashi,J. Hetzel,Nik Hirani,L. Homik,Ben Hope-Gill,D. Hotchkin,K. Ichikado,M. Ilkovich,Y. Inoue,S. Izumi,E. Jassem,L. Jones,Stéphane Jouneau,Robert J. Kaner,J. Kang,T. Kawamura,Romain Kessler,Y. Kim,K. Kishi,H. Kitamura,M. Kolb,Y. Kondoh,C. Kono,D Koschel,Michael Kreuter,Tejaswini Kulkarni,J. Kus,F. Lebargy,A. León Jiménez,Q. Luo,Yolanda Mageto,Toby M. Maher,S. Makino,Sylvain Marchand-Adam,C. Marquette,R. Martinez,M. Martínez,R. Maturana Rozas,Y. Miyazaki,Sergey Moiseev,Maria Molina-Molina,Lake Morrison,L. Morrow,T. Moua,Anoop M. Nambiar,Yasuhiko Nishioka,Hilario Nunes,M. Okamoto,Justin M. Oldham,Maria Otaola,Maria Padilla,J. S. Park,Nina Patel,Alberto Pesci,W. Piotrowski,L. Pitts,H. Poonyagariyagorn,Antje Prasse,Silvia Quadrelli,W. Randerath,R. Refini,M. Reynaud-Gaubert,F. Riviere,J. A. Rodríguez Portal,Ivan O. Rosas,M. Rossman,Zeenat Safdar,T. Saito,N. Sakamoto,M. Salinas Fénero,J. Sauleda,Shelley L. Schmidt,Mary Beth Scholand,M. Schwartz,Shane Shapera,Oksana A. Shlobin,B. Sigal,A. Silva Orellana,Dirk Skowasch,J. W. Song,S. Stieglitz,Helen Stone,Mary E. Strek,T. Suda,H. Sugiura,H. Takahashi,H. Takaya,T. Takeuchi,Krishna Thavarajah,Leslie Tolle,S. Tomassetti,K. Tomii,Claudia Valenzuela,Carlo Vancheri,F. Varone,Srihari Veeraraghavan,A. Villar,S. Weigt,L. Wemeau,W. Wuyts,Z. Xu,V. Yakusevich,Y. Yamada,H. Yamauchi,D. Ziora +167 more
TL;DR: The INBUILD trial suggests that nintedanib reduces the rate of ILD progression, as measured by FVC decline, in patients who have a chronic fibrosing ILD and progressive phenotype, irrespective of the underlying ILD diagnosis.
Journal ArticleDOI
Deep learning for classifying fibrotic lung disease on high-resolution computed tomography: a case-cohort study
TL;DR: This study investigated the use of a deep learning algorithm for provision of automated classification of fibrotic lung disease on high-resolution CT according to criteria specified in two international diagnostic guideline statements.
Journal ArticleDOI
What's in a name? That which we call IPF, by any other name would act the same.
TL;DR: The clinical and pathogenetic justification for a focus on “the progressive fibrotic phenotype” in future clinical and translational research is summarised, to address the needs of non-IPF patients with inexorably progressive Fibrotic disease, currently disenfranchised by lack of access to agents that are efficacious in IPF.
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An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias
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TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
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