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

Autor: Paul Sachs, Sally Suliman, Emily C. O'Brien, Kevin R. Flaherty, Tonya D. Russell, Timothy Liesching, Maryl Kreider, Joao Alberto de Andrade, Hyun J Kim, Andrew Namen, Justin M. Oldham, Daniel F. Dilling, Marilyn K. Glassberg, Shaun Bender, Zeenat Safdar, Francis Cordova, Rishi Raj, Wendy Morris, Scott M. Palmer, Barry Sigal, Randolph J. Lipchik, Albert Baker, Daniel A. Culver, Jason Lobo, Doug Lee, Rany Condos, Timothy P.M. Whelan, Anne S. Hellkamp, Thomas Leonard, Kalpalatha K. Guntupalli, Megan L. Neely, Rajat Walia, Joseph A. Lasky, Yolanda Mageto, Wael Asi, John A. Belperio, Scott Beegle, Laurie D. Snyder, Joao A. de Andrade, Amy Hajari Case, Nishant Gupta, Mridu Gulati, Craig S Conoscenti, David J. Lederer, Tristan J. Huie, Julie Fleming, Mary E. Strek, Prema Menon, Robert J. Kaner, Lisa Lancaster, Lake Morrison, Leann Silhan, Jeremy Tabak, Murali Ramaswamy, David Hotchkin
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Zdroj: Respiratory Research, Vol 20, Iss 1, Pp 1-10 (2019)
Respiratory Research
Popis: 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
Databáze: OpenAIRE
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