Prognostic factors in interstitial lung disease-associated pulmonary hypertension: data from the HYPID cohort and the French Pulmonary Hypertension Registry

BACKGROUND: Pulmonary hypertension (PH) frequently complicates interstitial lung diseases (ILDs), adversely affecting outcome. Identifying prognostic factors of patients with PH associated with ILD (ILD-PH) could facilitate early identification of patients who may benefit from PH therapy. METHODS: We included patients with ILD-PH from the prospective HYPID cohort and the French National Pulmonary Hypertension Registry (2007-2022). Univariable and multivariable analyses were performed to identify predictors of 1-year mortality. RESULTS: A total of 581 patients (mean age 69.4±9.3 years; 450 males) were analysed. ILD diagnoses were combined pulmonary fibrosis and emphysema (CPFE) syndrome (30.8%), idiopathic pulmonary fibrosis (29.6%), unclassifiable ILD (13.1%) and fibrotic hypersensitivity pneumonitis (10.3%). Mean pulmonary arterial pressure was 40.7±9.1 mmHg and mean pulmonary vascular resistance (PVR) was 7.6±3.5 Wood units (WU). Off-label PH therapy was initiated after initial evaluation in 215 patients (37%). The median transplant-free survival time was 17 (95% CI 15.2-not reached) months. Multivariable analysis identified male sex (p