Pulmonary hypertension in idiopathic pulmonary fibrosis: prevalence and clinical progress.

The aims of the present study are to define the prevalence of pulmonary hypertension (PH) in a cohort of idiopathic pulmonary fibrosis (IPF) patients, to investigate any correlations between systolic pulmonary artery pressure (PAPs) and functional data, to evaluate clinical progress and to compare long-term survival in IPF patients with and without PH. A population of 126 IPF patients was recruited. A high prevalence of PH (39.7%, 50/126), evaluated by echocardiography on the basis of PAPs greater than 36 mmHg, was mainly observed in smokers and female patients. Regression analysis revealed a significant correlation between PAPs greater than 50 mmHg and DLCO/VA (p = 0.0294). Mean PAPs was significantly greater one year after onset of PH (p = 0.01). 11/21 patients with FVC less than 50% had a significant increase in PAPs one year after onset of PH (p = 0.02). There was a highly significant difference between survival of IPF patients with and without PH (p = 0.0001; hazard ratio = 3.56). This study revealed that PH has a high prevalence in patients with IPF and is associated with increased risk of mortality. Early diagnosis of IPF patients with pulmonary hypertension is important, so that they can be enrolled in waiting lists for lung transplant as soon as possible.