Eur Respir J. 2026 Mar 26:2502233. doi: 10.1183/13993003.02233-2025. Online ahead of print.
ABSTRACT
BACKGROUND: Pulmonary hypertension (PH) frequently complicates interstitial lung diseases (ILD), adversely affecting outcome. Identifying prognostic factors of patients with 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 syndrome (30.8%), idiopathic pulmonary fibrosis (29.6%), unclassifiable ILD (13.1%), and fibrotic hypersensitivity pneumonitis (10.3%). Mean pulmonary artery pressure was 40.7±9.1 mmHg, and mean pulmonary vascular resistance was 7.6 ±3.5 Wood units. Off-label PH therapy was initiated after initial evaluation in 215 patients (37%). The median transplant-free survival time was 17 months (95% CI 15.2 - not reached). Multivariable analysis identified male sex (p<0.001), functional class III (p=0.003) or IV (p=0.003), 6 MWD≤228 m (p<0.001), PVR>5 Wood units (p=0.008), and absence of PH therapy (p<0.001) as independent predictors of death or lung transplantation at 1 year.
CONCLUSION: Non-invasive (6 MWD, WHO FC) and invasive (PVR) variables are associated with prognosis in patients with ILD-PH, including in patients with CPFE. PH medication might improve outcomes in this patient population.
PMID:41887671 | DOI:10.1183/13993003.02233-2025