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

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 po...

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Veröffentlicht in:Respirology (Carlton, Vic.) Vic.), 2018-06, Vol.23 (6), p.593-599
Hauptverfasser: 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.
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Sprache:eng
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Zusammenfassung: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. In combined pulmonary fibrosis and emphysema (CPFE), the likelihood of having pulmonary hypertension (PHT) is explained by the summed baseline computed tomography (CT) extents of interstitial lung disease and emphysema. There is no synergistic effect in CPFE increasing the likelihood of PHT. CPFE is therefore not associated with a malignant microvascular phenotype. See related Editorial
ISSN:1323-7799
1440-1843
DOI:10.1111/resp.13231