Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema

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Title: Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema
Authors: Jacob, J
Bartholmai, BJ
Rajagopalan, S
Karwoski, R
Nair, A
Walsh, SLF
Barnett, J
Cross, G
Judge, EP
Kokosi, M
Renzoni, E
Maher, TM
Wells, AU
Item Type: Journal Article
Abstract: BACKGROUND AND OBJECTIVE: This study evaluated whether patients with combined pulmonary fibrosis and emphysema (CPFE) have an increased likelihood of pulmonary hypertension (PHT) when compared with idiopathic pulmonary fibrosis (IPF) patients without emphysema. METHODS: Two consecutive IPF populations having undergone transthoracic echocardiography were examined (n = 223 and n = 162). Emphysema and interstitial lung disease (ILD) extent were quantified visually; ILD extent was also quantified by a software tool, CALIPER. Echocardiographic criteria categorized PHT risk. RESULTS: The prevalence of an increased PHT likelihood was 29% and 31% in each CPFE cohort. Survival at 12 months was 60% across both CPFE cohorts with no significantly worsened outcome identified when compared with IPF patients without emphysema. Using logistic regression models in both cohorts, total computed tomography (CT) disease extent (ILD and emphysema) predicted the likelihood of PHT. After adjustment for total disease extent, CPFE had no stronger association with PHT likelihood than IPF patients without emphysema. CONCLUSION: Our findings indicate that the reported association between CPFE and PHT is explained by the summed baseline CT extents of ILD and emphysema. Once baseline severity is taken into account, CPFE is not selectively associated with a malignant microvascular phenotype, when compared with IPF patients without emphysema.
Issue Date: 1-Jun-2018
Date of Acceptance: 15-Nov-2017
ISSN: 1323-7799
Publisher: Wiley
Start Page: 593
End Page: 599
Journal / Book Title: Respirology
Volume: 23
Issue: 6
Copyright Statement: © 2017 Asian Pacific Society of Respirology. This is the pre-peer reviewed version of the following article, which has been published in final form at
Sponsor/Funder: Arthritis Research UK
Funder's Grant Number: 20719
Keywords: Science & Technology
Life Sciences & Biomedicine
Respiratory System
computer tomography
idiopathic pulmonary fibrosis
pulmonary hypertension
11 Medical And Health Sciences
Publication Status: Published
Conference Place: Australia
Online Publication Date: 2017-12-13
Appears in Collections:National Heart and Lung Institute
Faculty of Medicine

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