@article{353396bb8f164ea0a88381412da47d86,
title = "Predictors of mortality in subjects with progressive fibrosing interstitial lung diseases",
abstract = "Background and objective: Demographic and clinical variables, measured at baseline or over time, have been associated with mortality in subjects with progressive fibrosing interstitial lung diseases (ILDs). We used data from the INPULSIS trials in subjects with idiopathic pulmonary fibrosis (IPF) and the INBUILD trial in subjects with other progressive fibrosing ILDs to assess relationships between demographic/clinical variables and mortality. Methods: The relationships between baseline variables and time-varying covariates and time to death over 52 weeks were analysed using pooled data from the INPULSIS trials and, separately, the INBUILD trial using a Cox proportional hazards model. Results: Over 52 weeks, 68/1061 (6.4%) and 33/663 (5.0%) subjects died in the INPULSIS and INBUILD trials, respectively. In the INPULSIS trials, a relative decline in forced vital capacity (FVC) >10% predicted within 12 months (hazard ratio [HR] 3.77) and age (HR 1.03 per 1-year increase) were associated with increased risk of mortality, while baseline FVC % predicted (HR 0.97 per 1-unit increase) and diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted (HR 0.77 per 1-unit increase) were associated with lower risk. In the INBUILD trial, a relative decline in FVC >10% predicted within 12 months (HR 2.60) and a usual interstitial pneumonia-like fibrotic pattern on HRCT (HR 2.98) were associated with increased risk of mortality, while baseline DLCO % predicted (HR 0.95 per 1-unit increase) was associated with lower risk. Conclusion: These data support similarity in the course of lung injury between IPF and other progressive fibrosing ILDs and the value of FVC decline as a predictor of mortality.",
keywords = "clinical trial, death, fibrosing interstitial lung disease, forced vital capacity, pulmonary fibrosis, pulmonary function test",
author = "Brown, {Kevin K.} and Yoshikazu Inoue and Flaherty, {Kevin R.} and Martinez, {Fernando J.} and Vincent Cottin and Francesco Bonella and Stefania Cerri and Danoff, {Sonye K.} and Stephane Jouneau and Goeldner, {Rainer Georg} and Martin Schmidt and Susanne Stowasser and Rozsa Schlenker-Herceg and Wells, {Athol U.}",
note = "Funding Information: Kevin K. Brown reports grants from NHLBI; has served on a Data Monitoring Committee for Biogen and Humanetics; has served as an advisor to AbbVie, Blade Therapeutics, Boehringer Ingelheim, Bristol‐Myers Squibb, CSL Behring, DevPro Biopharma, Dispersol, Galapagos, Galecto, Huitai Biomedicine, Lilly, the Open Source Imaging Consortium, Pliant, Redx Pharma, Sanofi, Theravance, Third Pole and Translate Bio; and reports scientific collaboration with Sekisui Medical Co. Yoshikazu Inoue reports grants from the Japan Agency for Medical Research and Development and the Japanese Ministry of Health, Labour, and Welfare; and payment for lectures or advisory boards from Asahi Kasei, Boehringer Ingelheim, Galapagos, Roche, Savara Inc., Shionogi & Co, Ltd and Taiho. Kevin R. Flaherty reports grants from Boehringer Ingelheim and Roche/Genentech and consulting fees from Bellerophon, Blade Therapeutics, Boehringer Ingelheim, DevPro, Horizon Pharmaceuticals, PureHealth, Respivant, Roche/Genentech, Shionogi & Co, Ltd and United Therapeutics. Fernando J. Martinez has served on a Data Safety Monitoring Board, advisory board or steering committee for Afferent/Merck, Bayer, Biogen, Boehringer Ingelheim, Nitto, Respivant, Roche and Veracyte; and has received consulting fees or payment for presentations from AbbVie, Boehringer Ingelheim, Bristol‐Myers Squibb, Bridge Biotherapeutics, CSL Behring, DevPro, IQVIA, Roche/Genentech, Sanofi, Shionogi & Co, Ltd, twoXAR, United Therapeutics and Veracyte. Vincent Cottin reports grants from Boehringer Ingelheim; consulting or lecturing fees from Actelion, AstraZeneca, Boehringer Ingelheim, Novartis, Roche/Promedior and Sanofi; support for attending meetings from Actelion, Boehringer Ingelheim and Roche/Promedior; and has served on a steering committee, advisory board, Data Safety Monitoring Board or adjudication committee for Actelion, Bayer/Merck Sharp & Dohme, Bristol‐Myers Squibb, Celgene, FibroGen, Galapagos, Galecto, Novartis, Roche/Promedior and Shionogi and Co, Ltd. Francesco Bonella has received speakers fees from Boehringer Ingelheim, Fujirebio, Galapagos and Roche; support for attending meetings from Boehringer Ingelheim and Roche; and has acted as an advisor for Boehringer Ingelheim, Bristol‐Myers Squibb, Fujirebio, Galapagos, GlaxoSmithKline, Roche and Takeda. Stefania Cerri reports payment for presentations from Boehringer Ingelheim. Sonye K. Danoff has been a clinical trial investigator or has served on a clinical trial coordinating committee for Boehringer Ingelheim, Bristol‐Myers Squibb and Roche/Genentech; has received consulting fees, presenting fees or support for attending meetings from Boehringer Ingelheim, the France Foundation and Lupin Pharma; has served on a Data Safety Monitoring Board or advisory board for Galecto and Galapagos; and has acted as an advisor for the American Thoracic Society and Pulmonary Fibrosis Foundation. Stephane Jouneau reports grants from AIRB, Boehringer Ingelheim, LVL, Novartis and Roche; payment for lectures or speaker bureaus from Actelion, AIRB, AstraZeneca, Boehringer Ingelheim, Bristol‐Myers Squibb, Chiesi, Genzyme, GlaxoSmithKline, LVL, MundiPharma, Novartis, Pfizer, Roche and Sanofi; support for attending meetings from Boehringer Ingelheim and Roche; and participation on advisory boards for Boehringer Ingelheim, Novartis and Roche. Rainer‐Georg Goeldner, Martin Schmidt and Susanne Stowasser are employees of Boehringer Ingelheim. Rozsa Schlenker‐Herceg was an employee of Boehringer Ingelheim at the time this research was conducted. Athol U. Wells reports consulting or speaker fees from Blade Therapeutics, Boehringer Ingelheim and Roche. Funding Information: : The INPULSIS and INBUILD trials were funded by Boehringer Ingelheim International GmbH. The authors did not receive payment for development of this article. Writing support was provided by Julie Fleming and Wendy Morris of Fleishman‐Hillard, London, UK, which was contracted and funded by Boehringer Ingelheim. Boehringer Ingelheim was given the opportunity to review the article for medical and scientific accuracy as well as intellectual property considerations. Research funding Publisher Copyright: {\textcopyright} 2022 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.",
year = "2022",
month = apr,
doi = "10.1111/resp.14231",
language = "English (US)",
volume = "27",
pages = "294--300",
journal = "Respirology",
issn = "1323-7799",
publisher = "Wiley-Blackwell",
number = "4",
}