Abstract
Background
Pulmonary hypertension (PH) complicates interstitial lung disease (ILD) and worsens patient outcomes; however, data beyond idiopathic pulmonary fibrosis (IPF) are limited. This study evaluated PH prevalence in progressive pulmonary fibrosis (PPF) and identified predictors of PH.Methods
Patients with ILD underwent echocardiography at Chiba University Hospital between January 2017 and December 2021 were screened (n = 144); five were excluded, yielding 139 patients. PH was defined by right heart catheterization (mean pulmonary artery pressure >20 mmHg) or by high echocardiographic PH probability according to 2022 ESC/ERS guidelines. PPF was classified using the 2022 guideline definition.Results
Seventy-six of 139 (54.7%) had PH. During the 3-year follow-up period, 40 patients (28.8%) died, and six underwent lung transplantation. Among the 61 patients with PPF and 47 with IPF, the prevalence of PH was similar (PPF 60.7% vs IPF 63.8%). No significant differences were observed in %FVC and %DLCO between the two groups. The PPF group included more females (57.4% vs 10.6%) and was younger (median 67 vs 74 years). In PPF, PH was not associated with a shorter survival (log-rank p = 0.565; HR 1.32, 95% CI 0.51-3.42). In PPF-PH, predictors included lower %FEV1 and %DLCO, higher BNP, ECG right-heart strain, and higher CT pulmonary artery/aorta ratio; multivariable analysis identified the artery/aorta ratio as the only independent factor, with a cutoff of 0.97 (sensitivity 75.7%, specificity 87.5%).Conclusions
PH is frequent in PPF at rates comparable to those in IPF, while its prognostic impact may be attenuated by heterogeneous mechanisms.Trial registration
This study was registered with the UMIN-CTR (UMIN000057743).