Abstract

BackgroundIdiopathic Pulmonary Fibrosis is a chronic, progressive interstitial lung disease for which there is no cure. However, lung function decline, hospitalizations, and mortality may be reduced with the use of the antifibrotic medications, nintedanib and pirfenidone. Historical outcomes for hospitalized patients with Idiopathic Pulmonary Fibrosis are grim; however there is a paucity of data since the approval of nintedanib and pirfenidone for treatment. In this study, we aimed to determine the effect of nintedanib and pirfenidone on mortality following respiratory-related hospitalizations, intensive care unit (ICU) admission, and mechanical ventilation.MethodsUsing a large U.S. insurance database, we created a one-to-one propensity score matched cohort of patients with idiopathic pulmonary fibrosis treated and untreated with an antifibrotic who underwent respiratory-related hospitalization between January 1, 2015 and December 31, 2018. Mortality was evaluated at 30 days and end of follow-up (up to 2 years). Subgroup analyses were performed for all patients receiving treatment in an ICU and those receiving invasive and non-invasive mechanical ventilation during the index hospitalization.ResultsAntifibrotics were not observed to effect utilization of mechanical ventilation or ICU treatment during the index admission or effect mortality at 30-days. If patients survived hospitalization, mortality was reduced in the treated cohort compared to the untreated cohort when followed up to two years (20.1% vs 47.8%).ConclusionsTreatment with antifibrotic medications does not appear to directly improve 30-day mortality during or after respiratory-related hospitalizations. Post-hospital discharge, however, ongoing antifibrotic treatment was associated with improved long-term survival.

Highlights

  • Idiopathic Pulmonary Fibrosis is a chronic, progressive interstitial lung disease for which there is no cure

  • We identified 274 treated and 2,039 untreated patients with Idiopathic Pulmonary Fibrosis (IPF), who had an intensive care unit (ICU) hospitalization between January 1, 2015 and December 31, 2018 and created a propensitymatched cohort for all ICU hospitalizations consisting of 274 matched pairs of treated and untreated patients (Table 2, Fig. 2)

  • Of those requiring an ICU hospitalization with mechanical ventilation (MV), we identified 94 treated and 751 untreated patients with IPF and created a propensity-matched cohort consisting of 94 matched pairs (Table 3, Fig. 2)

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Summary

Introduction

Idiopathic Pulmonary Fibrosis is a chronic, progressive interstitial lung disease for which there is no cure. Given lack of initial data to support a mortality benefit, debate was had as to whether these medications provided enough value for their use given the high costs of treatment and side effects; later data obtained from pooled analyses of the antifibrotic drug trials did suggest an additional mortality benefit [8,9,10,11] This data was complemented by that of an Australian IPF Registry [12], and more recently, administrative data from a large United States cohort of commercially insured and Medicare Advantage enrollees which demonstrated improved mortality on antifibrotic therapy [13]

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