Combined pulmonary fibrosis and emphysema: How does cohabitation affect respiratory functions?

Fatma Çiftci; Başak Gülpınar; Çetin Atasoy; Oya Kayacan; Sevgi Saryal

doi:10.1016/j.advms.2019.03.005

Combined pulmonary fibrosis and emphysema: How does cohabitation affect respiratory functions?

Adv Med Sci. 2019 Sep;64(2):285-291. doi: 10.1016/j.advms.2019.03.005. Epub 2019 Apr 1.

Authors

Fatma Çiftci¹, Başak Gülpınar², Çetin Atasoy², Oya Kayacan³, Sevgi Saryal³

Affiliations

¹ Department of Chest Disease, Ankara University School of Medicine, Ankara, Turkey. Electronic address: fciftci@ankara.edu.tr.
² Department of Radiology, Ankara University School of Medicine, Ankara, Turkey.
³ Department of Chest Disease, Ankara University School of Medicine, Ankara, Turkey.

PMID: 30947142
DOI: 10.1016/j.advms.2019.03.005

Abstract

Purpose: Combined pulmonary fibrosis and emphysema (CPFE) has emerged as a new syndrome with characteristics of both fibrosis and emphysema. We determined the impacts of radiologic emphysema severity on pulmonary function tests (PFTs), exercise capacity and mortality.

Patients and methods: IPF patients (n = 110) diagnosed at the Chest Diseases Clinic between September 2013 and January 2016 were enrolled in the study and followed up until June 2017. Visual and digital emphysema scores, PFTs, pulmonary artery pressure (sPAP), 6-minute walking test, composite physiologic index (CPI), and survival status were recorded. Patients with emphysema and those with pure IPF were compared.

Results: The CPFE-group had a significantly greater ratio of men(p < 0.001), lower BMI (p < 0.001), lower mean PaO₂ (p = 0.005), higher mean sPAP (p = 0.014), and higher exercise desaturation (p < 0.001). The CPFE group had a significantly higher FVC(L)(p = 0.016), and lower FEV1/FVC ratio (p = 0.002), DLCO, and DLCO/VA ratio(p = 0.03 and p = 0.005, respectively). Lung volumes of the CPFE group had significantly higher VC(p = 0.017), FRC (p < 0.001), RV(p < 0.001), RV/TLC(p < 0.001), and TLC(p < 0.001). There were significant correlations between emphysema scores and FVC (L)(p = 0.01), FEV1/FVC(p = 0.001), DLCO (p = 0.003), VC(p = 0.014), FRC (L)(p < 0.001), RV(p < 0.001), TLC(p < 0.001), and RV/TLC (p < 0.001). Mortality rates were comparable between the two groups. CPI (p = 0.02) and sPAP (p = 0.01) were independent predictors of mortality in patients with CPFE.

Conclusions: The presence and severity of emphysema affects pulmonary function in IPF. Patients with CPFE have reduced diffusion capacity, more severe air trapping, worse muscle weakness, more severe exercise desaturation, and pulmonary hypertension. CPI and pulmonary hypertension are two independent risk factors for mortality in subjects with CPFE.

Keywords: Combined pulmonary fibrosis and emphysema; Idiopathic pulmonary fibrosis; Mortality; Pulmonary function tests.

MeSH terms

Aged
Echocardiography
Female
Humans
Kaplan-Meier Estimate
Male
Middle Aged
Pulmonary Emphysema / metabolism
Pulmonary Emphysema / mortality*
Pulmonary Emphysema / pathology*
Pulmonary Fibrosis / metabolism
Pulmonary Fibrosis / mortality*
Pulmonary Fibrosis / pathology*
Respiratory Function Tests
Risk Factors