Assessment of Peripheral Endothelial Function in Idiopathic Pulmonary Fibrosis (Endoth-FPI)

The aim of the study is to assess the peripheral endothelial function in adult patients with idiopathic pulmonary fibrosis (IPF) and the relationship between the peripheral endothelial function and the severity of the IPF.

The primary criteria is the peripheral endothelial function that will be assessed by the measure of flow-mediated dilation (reactive hyperemia-peripheral artery tone index). The pulmonary function will be assessed by the measures of the forced expiratory volume at one second (FEV1), the forced vital capacity (FVC) and the total lung capacity (TLC) and the diffusing capacity of the lung for carbon monoxide (DLCO). The dyspnea will be assessed with the New York Heart Association (NYHA) score. The exercise capacity will be assessed by the 6-min walk test. Pulmonary arterial pressure will be recorded through cardiac echography or catheterization.

Inclusion Criteria: IPF according to ATS/ERS 2011's criteria Stable IPF (no reduction of FVC or DLCO of more than 10% and 15% respectively) Exclusion Criteria: Patients with a significant disease other than IPF. A significant disease is defined as a disease which, in the opinion of the investigator, may influence the results of the trial. Pregnant or nursing women. Non-pulmonary fibrosis treatment by pulmonary vasodilators that cannot be stopped for 24 hours for the assessment of endothelial function