Idiopathic pulmonary fibrosis (IPF) is a diffuse progressive parenchymal disease.
Patients with IPF have a poor prognosis and frequently suffer from pulmonary hypertension
(PH) complications, which are associated to significantly worse outcomes. In IPF, the
pathogenesis of PH varies and has not been completely understood yet, since it may arise
from multiple factors. In these patients, PH is associated with reduced exercise capacity and
lower survival rate. Due to its prognostic significance, it would be useful to conduct further
research into PH in IPF patients. Though right heart catheterization continues to be the gold standard diagnostic
test, the first steps to properly diagnose PH are clinical suspicion (based on increased shortness of breath
and reduced diffusing capacity) and echocardiography. The options to treat PH in this population are limited.
What is more, to date, there are no approved therapies specifically aimed at PH in IPF patients.