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 > 36 mmHg, was mainly observed in smokers and female patients. Regression analysis revealed a significant correlation between PAPs > 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 <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 patie...