Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry

Laurie Snyder; Megan L. Neely; Anne S. Hellkamp; Emily O'Brien; Joao De Andrade; Craig S. Conoscenti; Thomas Leonard; Shaun Bender; Mridu Gulati; Daniel A. Culver; Robert J. Kaner; Scott Palmer; Hyun Joo Kim; Wael Asi; Albert Baker; Scott Beegle; John A. Belperio; Rany Condos; Francis Cordova; Joao A.M. De Andrade; Daniel Dilling; Kevin Flaherty; Marilyn Glassberg; Kalpalatha Guntupalli; Nishant Gupta; Amy Hajari Case; David Hotchkin; Tristan Huie; 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; Sally Suliman; Jeremy Tabak; Rajat Walia; Timothy P. Whelan; Julie Fleming; Wendy Morris

doi:10.1186/s12931-019-1043-9

Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry

Laurie Snyder, Megan L. Neely, Anne S. Hellkamp, Emily O'Brien, Joao De Andrade, Craig S. Conoscenti, Thomas Leonard, Shaun Bender, Mridu Gulati, Daniel A. Culver, Robert J. Kaner, Scott Palmer, Hyun Joo Kim, Wael Asi, Albert Baker, Scott Beegle, John A. Belperio, Rany Condos, Francis Cordova, Joao A.M. De AndradeDaniel Dilling, Kevin Flaherty, Marilyn Glassberg, Kalpalatha Guntupalli, Nishant Gupta, Amy Hajari Case, David Hotchkin, Tristan Huie, 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, Sally Suliman, Jeremy Tabak, Rajat Walia, Timothy P. Whelan, Julie Fleming, Wendy Morris

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

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Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). Conclusions: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/. Trial registration: ClinicalTrials.gov number: NCT01915511.

Original language	English (US)
Article number	105
Journal	Respiratory Research
Volume	20
Issue number	1
DOIs	https://doi.org/10.1186/s12931-019-1043-9
State	Published - May 30 2019

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine

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10.1186/s12931-019-1043-9

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Snyder, L., Neely, M. L., Hellkamp, A. S., O'Brien, E., De Andrade, J., Conoscenti, C. S., Leonard, T., Bender, S., Gulati, M., Culver, D. A., Kaner, R. J., Palmer, S., Kim, H. J., Asi, W., Baker, A., Beegle, S., Belperio, J. A., Condos, R., Cordova, F., ... Morris, W. (2019). Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry. Respiratory Research, 20(1), Article 105. https://doi.org/10.1186/s12931-019-1043-9

Snyder, L, Neely, ML, Hellkamp, AS, O'Brien, E, De Andrade, J, Conoscenti, CS, Leonard, T, Bender, S, Gulati, M, Culver, DA, Kaner, RJ, Palmer, S, Kim, HJ, Asi, W, Baker, A, Beegle, S, Belperio, JA, Condos, R, Cordova, F, De Andrade, JAM, Dilling, D, Flaherty, K, Glassberg, M, Guntupalli, K, Gupta, N, Case, AH, Hotchkin, D, Huie, T, 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, Suliman, S, Tabak, J, Walia, R, Whelan, TP, Fleming, J & Morris, W 2019, 'Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry', Respiratory Research, vol. 20, no. 1, 105. https://doi.org/10.1186/s12931-019-1043-9

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title = "Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry",

abstract = "Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). Conclusions: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/. Trial registration: ClinicalTrials.gov number: NCT01915511.",

author = "Laurie Snyder and Neely, {Megan L.} and Hellkamp, {Anne S.} and Emily O'Brien and {De Andrade}, Joao and Conoscenti, {Craig S.} and Thomas Leonard and Shaun Bender and Mridu Gulati and Culver, {Daniel A.} and Kaner, {Robert J.} and Scott Palmer and Kim, {Hyun Joo} 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 Kevin Flaherty and Marilyn Glassberg and Kalpalatha Guntupalli and Nishant Gupta and Case, {Amy Hajari} and David Hotchkin and Tristan Huie 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 and Jeremy Tabak and Rajat Walia and Whelan, {Timothy P.} and Julie Fleming and Wendy Morris",

note = "Publisher Copyright: {\textcopyright} 2019 The Author(s).",

year = "2019",

month = may,

day = "30",

doi = "10.1186/s12931-019-1043-9",

language = "English (US)",

volume = "20",

journal = "Respiratory Research",

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TY - JOUR

T1 - Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis

T2 - Insights from the IPF-PRO Registry

AU - Snyder, Laurie

AU - Neely, Megan L.

AU - Hellkamp, Anne S.

AU - O'Brien, Emily

AU - De Andrade, Joao

AU - Conoscenti, Craig S.

AU - Leonard, Thomas

AU - Bender, Shaun

AU - Gulati, Mridu

AU - Culver, Daniel A.

AU - Kaner, Robert J.

AU - Palmer, Scott

AU - Kim, Hyun Joo

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

AU - Glassberg, Marilyn

AU - Guntupalli, Kalpalatha

AU - Gupta, Nishant

AU - Case, Amy Hajari

AU - Hotchkin, David

AU - Huie, Tristan

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

AU - Suliman, Sally

AU - Tabak, Jeremy

AU - Walia, Rajat

AU - Whelan, Timothy P.

AU - Fleming, Julie

AU - Morris, Wendy

PY - 2019/5/30

Y1 - 2019/5/30

N2 - Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). Conclusions: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/. Trial registration: ClinicalTrials.gov number: NCT01915511.

AB - Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. Methods: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. Results: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). Conclusions: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/. Trial registration: ClinicalTrials.gov number: NCT01915511.

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Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: Insights from the IPF-PRO Registry

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