Acute exacerbations of COPD versus IPF in patients with combined pulmonary fibrosis and emphysema

Massa Zantah,Yaniv Dotan,Hauqing Zhao,Chandra Dass,Nathaniel Marchetti,Gerard J Criner

doi:10.1186/s12931-020-01432-x

Abstract

RationalePatients with combined pulmonary fibrosis and emphysema (CPFE) may develop acute exacerbations of IPF (AE-IPF) or COPD (AE-COPD). The incidence and the characteristics of exacerbations in patients with CPFE (e.g., COPD vs IPF) have not been well described.ObjectivesTo compare the incidence and rate of exacerbations in patients with CPFE vs. IPF and evaluate their effect on clinical outcomes.MethodsComprehensive clinical data from CPFE and IPF patients were retrospectively reviewed. Baseline characteristics including lung function data, oxygen requirements, and pulmonary hemodynamics, were collected. Acute exacerbation events in both groups were defined clinically and radiographically. In the CPFE group, two patterns of exacerbations were identified. AE-COPD was defined clinically by symptoms of severe airflow obstruction causing respiratory failure and requiring hospitalization. Radiographic data were also defined based on previously published literature. AE-IPF was defined clinically as an acute hypoxic respiratory failure, requiring hospitalization and treatment with high dose corticosteroids. Radiographically, patients had to have a change in baseline imaging including presence of ground-glass opacities, interlobular septal thickening or new consolidations; that is not fully explained by other etiologies.ResultsEighty-five CPFE patients were retrospectively compared to 112 IPF patients. Of 112 patients with IPF; 45 had AE-IPF preceding lung transplant (40.18%) compared to 12 patients in the CPFE group (14.1%) (p < 0.05). 10 patients in the CPFE group experienced AE-COPD (11.7%). Patients with AE-IPF had higher mortality and more likely required mechanical ventilation and extracorporeal membrane oxygenation (ECMO) compared to patients with AE-COPD, whether their underlying disease was IPF or CPFE.ConclusionsCPFE patients may experience either AE-IPF or AE-COPD. Patients with CPFE and AE-COPD had better outcomes, requiring less intensive therapy compared to patients with AE-IPF regardless if underlying CPFE or IPF was present. These data suggest that the type of acute exacerbation, AE-COPD vs AE-IPF, has important implications for the treatment and prognosis of patients with CPFE.

Highlights

The association between pulmonary fibrosis and emphysema was initially described in 1990 by Wiggins et al, who described eight heavy smokers with pulmonary fibrosis and upper lobe emphysema on High-resolution computed tomography scans (HRCT)
Patients with combined pulmonary fibrosis and emphysema (CPFE) and Acute exacerbation (AE)-Chronic obstructive pulmonary disease (COPD) had better outcomes, requiring less intensive therapy compared to patients with AE-Idiopathic Pulmonary Fibrosis (IPF) regardless if underlying CPFE or IPF was present
These data suggest that the type of acute exacerbation, AE-COPD vs acute exacerbations of IPF (AE-IPF), has important implications for the treatment and prognosis of patients with CPFE

Summary

Introduction

The association between pulmonary fibrosis and emphysema was initially described in 1990 by Wiggins et al, who described eight heavy smokers with pulmonary fibrosis and upper lobe emphysema on High-resolution computed tomography scans (HRCT). These patients exhibited preserved lung volumes [1]. Several retrospective studies have contradicted this theory when they reported that the presence and extent of emphysema had no prognostic impact on survival of patients with IPF after correction for baseline disease severity [7, 8]

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