Premium
Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema
Author(s) -
Jacob Joseph,
Bartholmai Brian J.,
Rajagopalan Srinivasan,
Karwoski Ronald,
Nair Arjun,
Walsh Simon L.F.,
Barnett Joseph,
Cross Gary,
Judge Eoin P.,
Kokosi Maria,
Renzoni Elisabetta,
Maher Toby M.,
Wells Athol U.
Publication year - 2018
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13231
Subject(s) - medicine , idiopathic pulmonary fibrosis , pulmonary hypertension , interstitial lung disease , cardiology , cohort , pulmonary fibrosis , logistic regression , lung
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.