• Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Ronald Karwoski, Arjun Nair, Walsh Simon L F SLF Department of Radiology, Kings College Hospital NHS Foundation Trust, London, UK., Joseph Barnett, Gary Cross, Eoin P Judge, Maria Kokosi, Elisabetta Renzoni, Toby M Maher, and Athol U Wells.
• Division of Radiology, Mayo Clinic Rochester, Rochester, MN, USA.
• Respirology. 2018 Jun 1; 23 (6): 593-599.

Background And ObjectiveThis 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.MethodsTwo 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.ResultsThe 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.ConclusionOur 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.© 2017 Asian Pacific Society of Respirology.

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