Hypertension pulmonaire au cours des pneumopathies interstitielles diffuses

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure (mPAP) highter or equal to 25 mmHg. The diagnosis during a diffuse interstitial lung disease (PID) is made during a cardiac ultrasound, which, besides measuring the systolic pulmonary artery pressure, eliminate left heart failure and rank the probability of PH. Conventionally, this is a pre-capillary mechanism in this context. The frequency of PH in idiopathic pulmonary fibrosis (IPF) is from 8 to 50%, depending on the severity of the IPF. There is also a PH over the evolution of combined pulmonary fibrosis emphysema, sarcoidosis, histiocytosis, and more rarely lymphangioleiomyomatosis. Diagnostic confirmation requires the completion of a right heart catheterization performed in a competence or reference PH center. Treatment is based mainly on the management of respiratory disease and the introduction of long term oxygen therapy. Specific treatments for PAH are usually not indicated and can worsen gas exchange. Further studies are needed to determine the potential value of these treatments in patients with particularly severe PH.