Hospitalizations in patients with idiopathic pulmonary fibrosis

IPF-PRO Registry investigators, Hyun J. Kim, Laurie D. Snyder, Ayodeji Adegunsoye, Megan L. Neely, Shaun Bender, Eric S. White, Craig S. Conoscenti, Mary E. Strek

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Background: Hospitalizations are common among patients with idiopathic pulmonary fibrosis (IPF). We investigated the impact of hospitalizations on outcomes in patients with IPF. Methods: The IPF-PRO Registry is an observational US registry that enrolled patients with IPF that was diagnosed or confirmed at the enrolling center in the previous 6 months. Associations between patient characteristics and hospitalization, and between hospitalization and mortality, were analyzed using Cox regression models. Results: A total of 1002 patients with IPF were enrolled into the IPF-PRO Registry. Over a median follow-up time of 23.7 months (maximum: 67.0 months), 568 patients (56.7%) had at least one hospitalization. Of these patients, 319 (56.2%) had at least one respiratory-related hospitalization and 120 (21.1%) had at least one hospitalization with ventilatory support. Younger age (HR 0.68 [95% CI 0.55, 0.84] per 5-year increase for patients < 62 years), lower BMI (0.96 [0.93, 0.98] per 1-point increase), lower FVC % predicted (0.90 [0.83, 0.97] per 10% increase), oxygen use at rest (2.85 [2.18, 3.72]) and history of pulmonary hypertension (2.02 [1.37, 2.96]) at enrollment were associated with an increased risk of respiratory-related hospitalization during follow-up. In a multivariable model, there was an eightfold increase in the risk of mortality during hospitalization or within 90 days of discharge compared with outside of this period. The risk of mortality associated with a respiratory hospitalization or a hospitalization with ventilatory support was even greater. Conclusions: Data from the IPF-PRO Registry demonstrate that hospitalizations are common among patients with IPF. The risk of mortality during hospitalization or within 90 days of discharge was high, particularly among patients who were hospitalized for a respiratory cause or received ventilatory support. Trial registration, NCT01915511. Registered 5 August 2013,

Original languageEnglish (US)
Article number257
JournalRespiratory research
Issue number1
StatePublished - Dec 2021

Bibliographical note

Funding Information:
HJK is a member of the Publication Committee for the IPF-PRO/ILD-PRO Registry. LDS and MLN are faculty members in the Duke Clinical Research Institute (DCRI), which receives funding support from Boehringer Ingelheim Pharmaceuticals, Inc to co-ordinate the IPF-PRO/ILD-PRO Registry. AA reports grants and personal fees from Boehringer Ingelheim, personal fees from Genentech and grants from the Pulmonary Fibrosis Foundation and the National Institutes of Health. SB, ESW and CSC are employees of Boehringer Ingelheim Pharmaceuticals, Inc. MES reports grants, personal fees and non-financial support from Boehringer Ingelheim; grants from Galapagos; and personal fees from FibroGen.

Funding Information:
The IPF-PRO™ Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI) and co-ordinated by the Duke Clinical Research Institute (DCRI).

Funding Information:
The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE). The authors did not receive payment for development of this article. Writing support was provided by Julie Fleming and Wendy Morris of Fleishman-Hillard, London, UK, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. Boehringer Ingelheim was given the opportunity to review the article for medical and scientific accuracy as well as intellectual property considerations.

Publisher Copyright:
© 2021, The Author(s).


  • Interstitial lung disease
  • Mechanical ventilation
  • Mortality
  • Pulmonary fibrosis
  • Respiratory function tests

PubMed: MeSH publication types

  • Journal Article
  • Multicenter Study
  • Observational Study


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