Hospitalizations in patients with idiopathic pulmonary fibrosis
Authors: Kim Hyun J. , Snyder Laurie D. , Adegunsoye Ayodeji , Neely Megan L. , Bender Shaun , White Eric S. , Conoscenti Craig S. , Strek Mary E. ,   The IPF-PRO Registry Investigators, Baker Albert , Beegle Scott , Belperio John A. , Condos Rany , Cordova Francis , Culver Daniel A. , Dilling Daniel , Fitzgerald John , Silhan Leann , Flaherty Kevin R. , Gibson Kevin , Gulati Mridu , Guntupalli Kalpalatha , Gupta Nishant , Hajari Case Amy , Hotchkin David , Huie Tristan J. , Kaner Robert J. , Kim Hyun J. , Lancaster Lisa H. , Steele Mark , Lasky Joseph A. , Lee Doug , Liesching Timothy , Lipchik Randolph , Lobo Jason , Luckhardt Tracy R. , Andrade Joao A. , Mageto Yolanda , Huang Howard , Menon Prema , Mageto Yolanda , Morrison Lake , Namen Andrew , Oldham Justin M. , Paul Tessy , Zhang David , Podolanczuk Anna , Lederer David , Patel Nina M. , Porteous Mary , Kreider Maryl , Raj Rishi , Mohabir Paul , Ramaswamy Murali , Russell Tonya , Sachs Paul , Safdar Zeenat , Shafazand Shirin , Glassberg Marilyn , Siddiqi Ather , Asi Wael , Sigal Barry , Strek Mary E. , Noth Imre , Suliman Sally , Roman Jesse , Tabak Jeremy , Walia Rajat , Whelan Timothy P. M. 
Published: September 2021

Abstract
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 ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511