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

Journal Article (Journal Article)

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: . TRIAL REGISTRATION: number: NCT01915511 .

Full Text

Duke Authors

Cited Authors

  • 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; IPF-PRO™ Registry investigators,

Published Date

  • May 30, 2019

Published In

Volume / Issue

  • 20 / 1

Start / End Page

  • 105 -

PubMed ID

  • 31142314

Pubmed Central ID

  • PMC6542049

Electronic International Standard Serial Number (EISSN)

  • 1465-993X

Digital Object Identifier (DOI)

  • 10.1186/s12931-019-1043-9


  • eng

Conference Location

  • England