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

Publication ,  Journal Article
Snyder, L; Neely, ML; Hellkamp, AS; O'Brien, E; de Andrade, J; Conoscenti, CS; Leonard, T; Bender, S; Gulati, M; Culver, DA; Kaner, RJ ...
Published in: Respir Res
May 30, 2019

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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Published In

Respir Res

DOI

EISSN

1465-993X

Publication Date

May 30, 2019

Volume

20

Issue

1

Start / End Page

105

Location

England

Related Subject Headings

  • Respiratory System
  • Registries
  • Predictive Value of Tests
  • Mortality
  • Middle Aged
  • Male
  • Lung Transplantation
  • Idiopathic Pulmonary Fibrosis
  • Humans
  • Follow-Up Studies
 

Citation

APA
Chicago
ICMJE
MLA
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Snyder, L., Neely, M. L., Hellkamp, A. S., O’Brien, E., de Andrade, J., Conoscenti, C. S., … IPF-PROTM Registry investigators. (2019). Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res, 20(1), 105. https://doi.org/10.1186/s12931-019-1043-9
Snyder, Laurie, Megan L. Neely, Anne S. Hellkamp, Emily O’Brien, Joao de Andrade, Craig S. Conoscenti, Thomas Leonard, et al. “Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry.Respir Res 20, no. 1 (May 30, 2019): 105. https://doi.org/10.1186/s12931-019-1043-9.
Snyder L, Neely ML, Hellkamp AS, O’Brien E, de Andrade J, Conoscenti CS, et al. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res. 2019 May 30;20(1):105.
Snyder, Laurie, et al. “Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry.Respir Res, vol. 20, no. 1, May 2019, p. 105. Pubmed, doi: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, IPF-PROTM Registry investigators. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res. 2019 May 30;20(1):105.

Published In

Respir Res

DOI

EISSN

1465-993X

Publication Date

May 30, 2019

Volume

20

Issue

1

Start / End Page

105

Location

England

Related Subject Headings

  • Respiratory System
  • Registries
  • Predictive Value of Tests
  • Mortality
  • Middle Aged
  • Male
  • Lung Transplantation
  • Idiopathic Pulmonary Fibrosis
  • Humans
  • Follow-Up Studies