Background: Combined pulmonary fibrosis and emphysema (CPFE) is defined by
imaging findings and is characterized by the coexistence of both upper lobe emphysema and lower
lobe pulmonary fibrosis. The natural course of CPFE is frequently complicated by pulmonary arterial
hypertension (PAH), which identifies the most severe CPFE phenotype with a particularly poor
Case Report: In June 2013, a 78-year-old white male was admitted to the pulmonary rehabilitation
program because of exertional dyspnea. In 2007, he was diagnosed as CPFE due to imaging findings.
After diagnosis, the patient remained stable until 2011, when he started to get short of breath with
exertion. From September 2011, disease progression and worsening of the patient’s clinical condition
increased at a faster rate. During the hospitalization, a trans-thoracic two-dimensional
echocardiography showed the left ventricular chamber slightly dilated with an ejection fraction of
40% and systolic/diastolic/mean pulmonary arterial pressures on the right heart catheterization were
71/24/44 mmHg. Upon progressive clinical worsening, not due to worsening of lung function tests
but to the increase in PAH, an off-label use of ambrisentan was initiated as monotherapy and, then,
tadalafil was added. This therapy obtained an improvement in both clinical condition and exercise
capacity. Despite the advanced stage of lung disease, the patient survived for about 2 years after the
start of treatment with ambrisentan and tadalafil.
Conclusion: Some of the patients affected by CPFE complicated by PAH could benefit from the
addition of pulmonary vasodilator agents to conventional treatment.