Prevalence and predictors of pulmonary hypertension in patients with progressive pulmonary fibrosis: A retrospective study.

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).