@article{77663d4e8d68415584406494c02acd7e,
title = "Associations between patient-reported outcomes and death or lung transplant in idiopathic pulmonary fibrosis data from the idiopathic pulmonary fibrosis prospective outcomes registry",
abstract = "Rationale: Progression of idiopathic pulmonary fibrosis (IPF) is accompanied by worsening of symptoms, exercise capacity, and health-related quality of life. However, the utility of patient-reported outcomes as predictors of mortality remains uncertain. Objectives: To assess whether patient-reported outcomes are independently associated with mortality beyond clinical risk factors in patients with IPF. Methods: Data from the observational IPF Prospective Outcomes Registry were used to examine associations between patient-reported outcomes at enrollment and the composite outcome of death or lung transplant in the following year.vital capacity % predicted, and diffusing capacity of the lungs for carbon monoxide % predicted at enrollment). Results: Among 662 patients, 45 died and 12 underwent lung transplant over 1 year. In the model adjusted for age and clinical variables that were associated with death or lung transplant, worse scores on the St. George{\textquoteright}s Respiratory Questionnaire (SGRQ) total score (hazard ratio [HR], 1.22 [95% confidence interval (CI), 1.01–1.48] per 10-point increase), SGRQ activity score (HR, 1.25 [95% CI, 1.02–1.54] per 10-point increase) and SGRQ symptoms score (HR, 1.17 [95% CI, 1.01–1.36] per 10-point increase) were associated with death or lung transplant over 1 year. Conclusions: Patient-reported outcomes that assess symptoms and physical activity are independently associated with mortality in patients with IPF.",
keywords = "Interstitial lung diseases, Mortality, Observational study, Registries",
author = "{IPF-PRO Registry investigators} and Case, {Amy Hajari} and Hellkamp, {Anne S.} and Neely, {Megan L.} and Shaun Bender and Dilling, {Daniel F.} and Mridu Gulati and Hotchkin, {David L.} and Huie, {Tristan J.} and Lisa Lancaster and Snyder, {Laurie D.} and Conoscenti, {Craig S.} and Palmer, {Scott M.} and Wael Asi and Albert Baker and Scott Beegle and Belperio, {John A.} and Rany Condos and Francis Cordova and Culver, {Daniel A.} and Tracey Luckhardt and Flaherty, {Kevin R.} and Marilyn Glassberg and Kalpalatha Guntupalli and Nishant Gupta and David Hotchkin and Tristan Huie and Robert Kaner and Hyun Kim and Maryl Kreider and Lisa Lancaster and Joseph Lasky and David Lederer and Doug Lee and Timothy Liesching and Randolph Lipchik and Jason Lobo and Yolanda Mageto and Prema Menon and Lake Morrison and Andrew Namen and Justin Oldham and Rishi Raj and Murali Ramaswamy and Tonya Russell and Paul Sachs and Zeenat Safdar and Barry Sigal and Leann Silhan and Mary Strek and Sally Suliman",
note = "Funding Information: Supported by Boehringer Ingelheim Pharmaceuticals, Inc. funds to the Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry and coordinated by the Duke Clinical Research Institute. Writing support was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc., which was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations. Publisher Copyright: Copyright {\textcopyright} 2020 by the American Thoracic Society",
year = "2020",
month = jun,
doi = "10.1513/AnnalsATS.201906-437OC",
language = "English (US)",
volume = "17",
pages = "699--705",
journal = "Annals of the American Thoracic Society",
issn = "2325-6621",
publisher = "American Thoracic Society",
number = "6",
}