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 Dilling; Kevin 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

doi:10.1016/j.chest.2019.11.042

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

Medicine - Pulmonary, Allergy, Critical

Research output: Contribution to journal › Article › peer-review

20 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	1188-1198
Number of pages	11
Journal	CHEST
Volume	157
Issue number	5
DOIs	https://doi.org/10.1016/j.chest.2019.11.042
State	Published - 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

Keywords

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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IPF-PRO Registry investigators, O'Brien, E. C., Hellkamp, A. S., Neely, M. L., Swaminathan, A., Bender, S., Snyder, L. D., Culver, D. A., Conoscenti, C. S., Todd, J. L., Palmer, S. M., Leonard, T. B., Asi, W., Baker, A., Beegle, S., Belperio, J. A., Condos, R., Cordova, F., de Andrade, J. A. M., ... Strek, M. (2020). Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry. CHEST, 157(5), 1188-1198. https://doi.org/10.1016/j.chest.2019.11.042

IPF-PRO Registry investigators, O'Brien, EC, Hellkamp, AS, Neely, ML, Swaminathan, A, Bender, S, Snyder, LD, Culver, DA, Conoscenti, CS, Todd, JL, Palmer, SM, Leonard, TB, Asi, W, Baker, A, Beegle, S, Belperio, JA, Condos, R, Cordova, F, de Andrade, JAM, Dilling, D, Flaherty, KR, Glassberg, M, Gulati, M, Guntupalli, K, Gupta, N, Hajari Case, A, Hotchkin, D, Huie, T, Kaner, R, Kim, H, Kreider, M, Lancaster, L, Lasky, J, Lederer, D, Lee, D, Liesching, T, Lipchik, R, Lobo, J, Mageto, Y, Menon, P, Morrison, L, Namen, A, Oldham, J, Raj, R, Ramaswamy, M, Russell, T, Sachs, P, Safdar, Z, Sigal, B, Silhan, L & Strek, M 2020, 'Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A Cross-Sectional Analysis of the IPF-PRO Registry', CHEST, vol. 157, no. 5, pp. 1188-1198. https://doi.org/10.1016/j.chest.2019.11.042

@article{8518a6bad6584fa8aab3d142bea9f03d,

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

abstract = "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.",

keywords = "idiopathic pulmonary fibrosis, lung function, quality of life",

author = "{IPF-PRO Registry investigators} and O'Brien, {Emily C.} and Hellkamp, {Anne S.} and Neely, {Megan L.} and Aparna Swaminathan and Shaun Bender and Snyder, {Laurie D.} and Culver, {Daniel A.} and Conoscenti, {Craig S.} and Todd, {Jamie L.} and Palmer, {Scott M.} and Leonard, {Thomas B.} and Wael Asi and Albert Baker and Scott Beegle and Belperio, {John A.} and Rany Condos and Francis Cordova and {de Andrade}, {Joao A.M.} and Daniel Dilling and Flaherty, {Kevin R.} and Marilyn Glassberg and Mridu Gulati and Kalpalatha Guntupalli and Nishant Gupta and {Hajari Case}, Amy 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",

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{\textquoteright}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: {\textcopyright} 2020 The Authors",

year = "2020",

month = may,

doi = "10.1016/j.chest.2019.11.042",

language = "English (US)",

volume = "157",

pages = "1188--1198",

journal = "CHEST",

issn = "0012-3692",

publisher = "American College of Chest Physicians",

number = "5",

}

TY - JOUR

T1 - Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis

T2 - A Cross-Sectional Analysis of the IPF-PRO Registry

AU - IPF-PRO Registry investigators

AU - O'Brien, Emily C.

AU - Hellkamp, Anne S.

AU - Neely, Megan L.

AU - Swaminathan, Aparna

AU - Bender, Shaun

AU - Snyder, Laurie D.

AU - Culver, Daniel A.

AU - Conoscenti, Craig S.

AU - Todd, Jamie L.

AU - Palmer, Scott M.

AU - Leonard, Thomas B.

AU - Asi, Wael

AU - Baker, Albert

AU - Beegle, Scott

AU - Belperio, John A.

AU - Condos, Rany

AU - Cordova, Francis

AU - de Andrade, Joao A.M.

AU - Dilling, Daniel

AU - Flaherty, Kevin R.

AU - Glassberg, Marilyn

AU - Gulati, Mridu

AU - Guntupalli, Kalpalatha

AU - Gupta, Nishant

AU - Hajari Case, Amy

AU - Hotchkin, David

AU - Huie, Tristan

AU - Kaner, Robert

AU - Kim, Hyun

AU - Kreider, Maryl

AU - Lancaster, Lisa

AU - Lasky, Joseph

AU - Lederer, David

AU - Lee, Doug

AU - Liesching, Timothy

AU - Lipchik, Randolph

AU - Lobo, Jason

AU - Mageto, Yolanda

AU - Menon, Prema

AU - Morrison, Lake

AU - Namen, Andrew

AU - Oldham, Justin

AU - Raj, Rishi

AU - Ramaswamy, Murali

AU - Russell, Tonya

AU - Sachs, Paul

AU - Safdar, Zeenat

AU - Sigal, Barry

AU - Silhan, Leann

AU - Strek, Mary

N1 - 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

PY - 2020/5

Y1 - 2020/5

N2 - 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.

AB - 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.

KW - idiopathic pulmonary fibrosis

KW - lung function

KW - quality of life

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

Abstract

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