Pulmonary hypertension risk in CPFE linked to summed extents of ILD, emphysema

Patients with combined pulmonary fibrosis and emphysema (CPFE) are likely to develop pulmonary hypertension, and this likelihood may be explained by the summed extents of interstitial lung disease (ILD) and emphysema, according to a study.

The study included two consecutive CPFE populations having undergone transthoracic echocardiography (n=223 and n=162). Researchers quantified emphysema and ILD extent, as well as categorized the risk of pulmonary hypertension using echocardiographic criteria.

The prevalence of an increased likelihood of pulmonary hypertension was 29 and 31 percent in each CPFE cohort. At 12 months, survival was 60 percent across both cohorts, with no significantly worsened outcome observed when compared with idiopathic pulmonary fibrosis (IPF) patients without emphysema.

In logistic regression models, total computed tomography disease extent (ILD and emphysema) was found to predict the likelihood of pulmonary hypertension.

After adjustment for total disease extent, CPFE had no stronger association with PHT likelihood than IPF patients without emphysema.

When ILD extent and visual emphysema extent were examined in CPFE patients in both cohorts, ILD and emphysema scores had a similar impact on the likelihood of PHT. In other words, the likelihood of pulmonary hypertension was not significantly increased in CPFE patients than in IPF patients without emphysema.

“There is no synergistic effect in CPFE increasing the likelihood of pulmonary hypertension,” researchers noted. “Accordingly, with regard to the likelihood of having pulmonary hypertension, CPFE is not greater than the sum of its parts (ILD and emphysema) and is not associated with a malignant microvascular phenotype.”