Abstract

BackgroundIdiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations. Studies suggest mechanical ventilation (MV) use in IPF does not improve outcomes and guidelines recommend against its general use. Our objective was to investigate MV use and association with cost and mortality in IPF.MethodsThis retrospective study, using a nationwide sample, included claims with IPF (ICD-9-CM: 516.3) in 2009–2011 and principal respiratory disease diagnosis (ICD-9-CM: 460–519); excluding lung transplant. Regression models were used to determine predictors of MV and association with cost, LOS, and mortality. Domain analysis was used to account for use of subpopulation. Costs were adjusted to 2011. Data on patient severity not available.ResultsTwenty two thousand three hundred fifty non-transplant IPF patients were admitted with principal respiratory disease diagnosis: Mean age 70.0 (SD 13.9), 49.1% female, mean LOS 7.4 (SD 8.2). MV was used in 11.4% of patients with a non-significant decline over time. In regression models, MV was associated with an increased stay of 9.78 days (95% CI 8.38–11.18) and increased cost of $36,583 (95% CI $32,021–41,147). MV users had significantly increased mortality (OR 15.55, 95% CI 12.13–19.95) versus nonusers.ConclusionsMechanical ventilation use has not significantly changed over time and is mostly used in younger patients and those admitted for non-IPF respiratory conditions. MV was associated with a 4-fold admission cost increase ($49,924 versus $11,742) and a 7-fold mortality increase (56% versus 7.5%), although patients who receive MV may differ from those who do not. Advances in treatment and decision aids are needed to improve outcomes in IPF.

Highlights

  • Idiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations

  • We looked for evidence of concomitant acute and chronic pulmonary conditions, including chronic obstructive pulmonary disease (COPD), bacterial pneumonia, and lung

  • From 2009–2011 42,924 IPF patients were admitted to US short-stay hospitals; 23,739 admissions had a principal diagnosis of respiratory disease

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Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations. Studies suggest mechanical ventilation (MV) use in IPF does not improve outcomes and guidelines recommend against its general use. Management of respiratory failure in IPF is challenging as patients can develop acute disease episodes that necessitate ventilator support. A systematic review [17] summarizing 9 single-center studies reported an 87% in-hospital mortality rate for IPF patients who received MV. Given this evidence, IPF treatment guidelines recommend the majority of IPF patients with respiratory failure not receive MV, and when used should occur

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