Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry

IPF-PRO Registry investigators, Emily C. O'Brien, Anne S. Hellkamp, Megan L. Neely, Aparna Swaminathan, Shaun Bender, Laurie D. Snyder, Daniel A. Culver, Craig S. Conoscenti, Jamie L. Todd, Scott M. Palmer, Thomas B. Leonard, Wael Asi, Albert Baker, Scott Beegle, John A. Belperio, Rany Condos, Francis Cordova, Joao A.M. de Andrade, Daniel DillingKevin R. Flaherty, Marilyn Glassberg, Mridu Gulati, Kalpalatha Guntupalli, Nishant Gupta, Amy Hajari Case, David Hotchkin, Tristan Huie, Robert Kaner, Hyun Kim, Maryl Kreider, Lisa Lancaster, Joseph Lasky, David Lederer, Doug Lee, Timothy Liesching, Randolph Lipchik, Jason Lobo, Yolanda Mageto, Prema Menon, Lake Morrison, Andrew Namen, Justin Oldham, Rishi Raj, Murali Ramaswamy, Tonya Russell, Paul Sachs, Zeenat Safdar, Barry Sigal, Leann Silhan, Mary Strek

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Background: Limited data are available on the association between clinically measured disease severity markers and quality of life (QOL) in idiopathic pulmonary fibrosis (IPF). The study examined the associations between objective disease severity metrics and QOL in a contemporary IPF population. Methods: This study evaluated baseline data from patients enrolled in the multicenter, US-based Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry between June 2014 and July 2018. Disease severity metrics included FVC % predicted, diffusing capacity for carbon monoxide (DLCO) % predicted, supplemental oxygen use with activity, supplemental oxygen use at rest, and two summary scores (the Gender-Age-Lung Physiology index, based on gender, age, and % predicted values for DLCO and FVC; and the Composite Physiologic Index, based on % predicted values for DLCO, FVC, and FEV1). Multivariable adjusted regression models were used to examine cross-sectional associations between each severity measure and St. George's Respiratory Questionnaire (SGRQ) total score. Results: Among 829 patients with complete SGRQ data, the median (interquartile range) SGRQ score at enrollment was 40 (26-53), with higher scores indicating worse QOL. Modest SGRQ impairments were observed with increasing Gender-Age-Lung Physiology score (2.9 [1.8-4.0] per 1-point increase] and with increasing Composite Physiologic Index scores (3.0 [2.4-3.6] per 5-point increase). Substantial SGRQ impairments were observed for oxygen use with activity (15.6 [12.9-18.2]), oxygen use at rest (16.2 [13.0-19.4]), and decreasing DLCO (5.0 [4.0-6.1] per 10% decrease in % predicted). Conclusions: Objective measures of disease severity, including severity scores, physiologic parameters, and supplemental oxygen use, are associated with worse QOL in patients with IPF. Trial Registry: ClinicalTrials.gov; No.: NCT01915511; URL: www.clinicaltrials.gov.

Original languageEnglish (US)
Pages (from-to)1188-1198
Number of pages11
Issue number5
StatePublished - May 2020

Bibliographical note

Funding Information:
Other contributions: The authors acknowledge the IPF-PRO registry principal investigators: Wael Asi, Renovatio Clinical, The Woodlands, TX; Albert Baker, Lynchburg Pulmonary Associates, Lynchburg, VA; Scott Beegle, Albany Medical Center, Albany, NY; John A. Belperio, University of California Los Angeles, Los Angeles, CA; Rany Condos, NYU Medical Center, New York, NY; Francis Cordova, Temple University, Philadelphia, PA; Daniel A. Culver, Cleveland Clinic, Cleveland, OH; Joao A.M. de Andrade, then at University of Alabama at Birmingham, Birmingham, AL; Daniel Dilling, Loyola University Health System, Maywood, IL; Kevin R. Flaherty, University of Michigan, Ann Arbor, MI; Marilyn Glassberg, University of Miami, Miami, FL; Mridu Gulati, Yale School of Medicine, New Haven, CT; Kalpalatha Guntupalli, Baylor College of Medicine, Houston, TX; Nishant Gupta, University of Cincinnati Medical Center, Cincinnati, OH; Amy Hajari Case, Piedmont Healthcare, Austell, GA; David Hotchkin, The Oregon Clinic, Portland, OR; Tristan Huie, National Jewish Hospital, Denver, CO; Robert Kaner, Weill Cornell Medical College, New York, NY; Hyun Kim, University of Minnesota, Minneapolis, MN; Maryl Kreider, University of Pennsylvania, Philadelphia, PA; Lisa Lancaster, Vanderbilt University, Nashville, TN; Joseph Lasky, Tulane University, New Orleans, LA; David Lederer, Columbia University Medical Center/New York Presbyterian Hospital, New York, NY; Doug Lee, Wilmington Health and PMG Research, Wilmington, NC; Timothy Liesching, Lahey Clinic, Burlington, MA; Randolph Lipchik, Froedtert & The Medical College of Wisconsin Community Physicians, Milwaukee, WI; Jason Lobo, UNC Chapel Hill, Chapel Hill, NC; Yolanda Mageto, Baylor University Medical Center at Dallas, Dallas, TX; Prema Menon, Vermont Lung Center, Colchester, VT; Lake Morrison, Duke University Medical Center, Durham, NC; Andrew Namen, Wake Forest University, Winston Salem, NC; Justin Oldham, University of California, Davis, Sacramento, CA; Rishi Raj, Stanford University, Stanford, CA; Murali Ramaswamy, PulmonIx LLC, Greensboro, NC; Tonya Russell, Washington University, St. Louis, MO; Paul Sachs, Pulmonary Associates of Stamford, Stamford, CT; Zeenat Safdar, Houston Methodist Lung Center, Houston, TX; Barry Sigal, Salem Chest and Southeastern Clinical Research Center, Winston Salem, NC; Leann Silhan, UT Southwestern Medical Center, Dallas, TX; Mary Strek, University of Chicago, Chicago, IL; Sally Suliman, University of Louisville, Louisville, KY; Jeremy Tabak, South Miami Hospital, South Miami, FL; Rajat Walia, St. Joseph’s Hospital, Phoenix, AZ; and Timothy P. Whelan, Medical University of South Carolina, Charleston, SC. Editorial support was provided by Wendy Morris, MSc, of FleishmanHillard Fishburn, London, UK, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc.

Publisher Copyright:
© 2020 The Authors


  • idiopathic pulmonary fibrosis
  • lung function
  • quality of life

PubMed: MeSH publication types

  • Journal Article
  • Multicenter Study
  • Research Support, Non-U.S. Gov't


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