Abstract
BackgroundHospitalizations are common among patients with idiopathic pulmonary fibrosis (IPF). We investigated the impact of hospitalizations on outcomes in patients with IPF.MethodsThe 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.ResultsA 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.ConclusionsData 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 ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511
Highlights
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial lung disease associated with progressive decline in lung function and a poor prognosis [1]
Lower body mass index (BMI), lower forced vital capacity (FVC) % predicted, oxygen use at rest and history of pulmonary hypertension at enrollment were associated with an increased risk of respiratory-related hospitalization during follow-up (Table 4)
Younger age, lower BMI, lower FVC, oxygen use at rest, and a history of pulmonary hypertension at enrollment were associated with an increased risk of respiratory-related hospitalization
Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial lung disease associated with progressive decline in lung function and a poor prognosis [1]. Hospitalizations are common among patients with IPF and are. The Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry (NCT01915511) is a multicenter, observational registry of patients with IPF in the US that aims to improve understanding of the clinical course and impact of IPF and current practices in its diagnosis and care [7]. Kim et al Respir Res (2021) 22:257 hospitalizations in patients with IPF have been based on databases of insurance claims or electronic medical records. Observational registries provide the opportunity to investigate the risk and impact of hospitalizations in clinical practice. Hospitalizations are common among patients with idiopathic pulmonary fibrosis (IPF). We investigated the impact of hospitalizations on outcomes in patients with IPF
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