Abstract

BackgroundPrevious analyses of combined pulmonary fibrosis and emphysema (CPFE) cohorts have provided conflicting data on the survival of patients with CPFE. Therefore, the aim of this study was to investigate the clinical prognosis of acute exacerbations (AE) of CPFE.MethodsWe retrospectively reviewed the medical records of patients who had been treated at the Shinshu University Hospital (Matsumoto, Japan) between 2003 and 2017. We identified 21 patients with AE of CPFE and 41 patients with AE of idiopathic pulmonary fibrosis (IPF) and estimated their prognoses using the Kaplan–Meier method.ResultsTreatment content and respiratory management were not significantly different between the two groups before and after exacerbation. At the time of AE, the median serum Krebs von den Lungen-6 level was significantly lower in the CPFE group (Krebs von den Lungen-6: 966 U/μL; white blood cell count: 8810 /μL) than that in the IPF group (Krebs von den Lungen-6: 2130 U/μL, p < 0.001; white blood cells: 10809/μL, p = 0.0096). The baseline Gender-Age-Physiology scores were not significantly different between the two groups (CPFE, 4.5 points; IPF, 4.7 points; p = 0.58). Kaplan–Meier curves revealed that the survival time after AE for patients with CPFE was longer than that for patients with IPF (p < 0.001, log-rank test).ConclusionsSurvival prognoses after AE were significantly better for patients with CPFE than that for those with IPF. Our findings may improve the medical treatment and respiratory management of patients with AE-CPFE.

Highlights

  • Previous analyses of combined pulmonary fibrosis and emphysema (CPFE) cohorts have provided conflicting data on the survival of patients with CPFE

  • Study design We retrospectively reviewed the medical records of patients with CPFE and idiopathic pulmonary fibrosis (IPF), who had been treated at the Shinshu University Hospital (Matsumoto, Japan) between 2003 and 2017

  • Baseline clinical characteristics and data of patients with CPFE and IPF Based on the selection criteria described previously, the clinical data of 21 patients with acute exacerbations (AE)-CPFE and 41 patients with AE-IPF were enrolled in the present study

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Summary

Introduction

Previous analyses of combined pulmonary fibrosis and emphysema (CPFE) cohorts have provided conflicting data on the survival of patients with CPFE. Previous analyses of CPFE cohorts have provided conflicting data on the survival. Mejia et al [7] reported that the overall prognosis in patients who had CPFE with the usual interstitial pneumonia (UIP) pattern was poorer than that of patients. Kurashima et al [4] reported that the overall prognosis was better for patients with CPFE than that for those with IPF. They showed that 31.8% of patients died because of AE-IPF in contrast to 11.1% who died because of AE-CPFE. We compared differences in the prognosis between patients with AE-CPFE and those with AE-IPF

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