Hospitalizations in patients with idiopathic pulmonary fibrosis

Respir Res. 2021 Sep 30;22(1):257. doi: 10.1186/s12931-021-01851-4.


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,

Keywords: Interstitial lung disease; Mechanical ventilation; Mortality; Pulmonary fibrosis; Respiratory function tests.

Publication types

  • Multicenter Study
  • Observational Study

MeSH terms

  • Aged
  • Female
  • Hospital Mortality
  • Hospitalization*
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnosis
  • Idiopathic Pulmonary Fibrosis / mortality
  • Idiopathic Pulmonary Fibrosis / therapy*
  • Male
  • Patient Discharge
  • Patient Readmission
  • Prognosis
  • Registries
  • Respiration, Artificial / adverse effects*
  • Respiration, Artificial / mortality
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • United States

Associated data