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 - 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
UR - http://www.scopus.com/inward/record.url?scp=85081949949&partnerID=8YFLogxK
U2 - 10.1016/j.chest.2019.11.042
DO - 10.1016/j.chest.2019.11.042
M3 - Article
C2 - 31954102
AN - SCOPUS:85081949949
SN - 0012-3692
VL - 157
SP - 1188
EP - 1198
JO - Chest
JF - Chest
IS - 5
ER -