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 journalArticle

8 Scopus citations

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 languageEnglish (US)
Article number105
JournalRespiratory Research
Volume20
Issue number1
DOIs
StatePublished - May 30 2019

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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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), [105]. https://doi.org/10.1186/s12931-019-1043-9