Rev Med Liege. 2026 Oct;81(10):589-596.
ABSTRACT
Pulmonary hypertension associated with interstitial lung diseases (PH-ILD) is a common and severe complication of interstitial lung diseases. Diagnosis is challenging and relies on a combination of clinical, functional, biological, radiological and echocardiographic findings. Definitive diagnosis is established by right heart catheterisation, which confirms the presence of pre-capillary pulmonary hypertension associated with increased pulmonary vascular resistance. Until 2021, and the INCREASE study, all randomised controlled trials evaluating therapies approved for pulmonary arterial hypertension in patients with PH-ILD yielded disappointing results. However, recent advances exploring the potential of novel therapeutic strategies have renewed interest in PH-ILD, with a progressive paradigm shift opening new perspectives for a condition that was until recently associated with a poor prognosis.
PMID:42861537