Pulmonary hypertension optimal screening in interstitial lung disease (PHOS-ILD): a scoring system for the detection of pulmonary hypertension in patients with interstitial lung disease.
- 1Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Ubben Center for Pulmonary Fibrosis Research, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.
- 2First Department of Pulmonary, Critical Care and Sleep Medicine, Hospital for Thoracic Diseases "SOTIRIA," National and Kapodistrian University of Athens, Athens, Greece.
- 3Division of Pulmonary and Critical Care Medicine, Integrated Hospital Care Institute, Cleveland Clinic, Cleveland, OH, United States.
Abstract
Background
Pulmonary hypertension (PH) is a common comorbidity of interstitial lung disease (ILD). Timely detection may improve outcomes through implementation of PH specific treatments.Methods
Retrospective, multicenter study aiming to identify features that predict PH in patients with ILD. In the derivation cohort (ILD cohort-University of South Florida), we identified predictors of PH in ILD and created a scoring system using the rounded value of odds ratio for each significant parameter. Correlation with hemodynamic parameters was investigated in the derivation and validation cohort (Idiopathic Pulmonary Fibrosis cohort-Cleveland Clinic).Results
We included 164 patients with ILD that underwent right heart catheterization (RHC) [derivation cohort: n = 118, median age (years, 95% CI): 69.0 (65.9-71.0), female gender: 65 (54.6%), median mean pulmonary arterial pressure (mPAP) (mmHg, 95% CI): 25.0 (23.0-27.0), median pulmonary vascular resistance (PVR) [Wood units (WU), 95% CI]: 3.6 (3.0-4.3), validation cohort: n = 46, median age (95% CI): 63.0 (60.0-66.0), female gender: 17 (37%), median mPAP (mmHg, 95% CI): 20.5 (18.0-27.2), median PVR (WU, 95% CI): 3.1 (2.2-3.4)]. Parameters and optimal cut-offs that significantly predicted precapillary PH in the derivation cohort were: age>42 years, heart rate recovery (HRR) at 1 min ≤ 15 bpm in 6-min walk test (6 MWT), 6-min walk distance (6 MWD) ≤ 270 meters, B-type natriuretic peptide (BNP) > 136 pg/mL, pulmonary artery to ascending aorta diameter ratio (PA/AA) > 1 in high-resolution computed tomography (HRCT), main PA diameter > 29 mm in HRCT, right ventricular systolic pressure > 42 mmHg on echocardiogram. We created a PH detection model that ranged from 0 to 28. The optimal cut-off threshold for the detection of PH was > 14 (80% sensitivity, 68% specificity, AUC: 0.78). A simplified scoring system without BNP and main PA diameter (range: 0-21) maintained the same AUC. The simplified scoring system exhibited positive correlation with mean pulmonary artery pressure both in the derivation (rho = 0.47, p < 0.0001) and validation cohort (rho = 0.36, p = 0.013).Conclusion
This study demonstrates that incorporation of demographic, functional, imaging, and echocardiographic characteristics can optimize the early identification of precapillary PH in patients with ILD, paving the way to structured screening strategies. Timely detection of PH might lead to timely implementation of emerging PH specific treatments in patients with ILDs.
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BibTeX
@article{karampitsakos2026pulmonary,
title = {{Pulmonary hypertension optimal screening in interstitial lung disease (PHOS-ILD): a scoring system for the detection of pulmonary hypertension in patients with interstitial lung disease.}},
author = {Theodoros Karampitsakos and Evan Wasserman and Eleni Stagaki and Abhay Thottassery and Jorden Smith and Huda Asif and Brian Soto and Christian Arce Guzman and Jalen Hammonds and Anna Papasarantou and Agamemnon Bakakos and Ourania Papaioannou and Apostolos Matsioulas and Foteini Karakontaki and Konstantinos Loverdos and Eirini Kefalidou and Brenda M Juan-Guardela and Jose D Herazo-Maya and Adriano R Tonelli and Debabrata Bandyopadhyay},
journal = {Frontiers in medicine},
year = {2026},
month = oct,
doi = {10.3389/fmed.2026.1908302},
url = {https://europepmc.org/article/MED/42898971},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pulmonary hypertension optimal screening in interstitial lung disease (PHOS-ILD): a scoring system for the detection of pulmonary hypertension in patients with interstitial lung disease.
AU - Theodoros Karampitsakos
AU - Evan Wasserman
AU - Eleni Stagaki
AU - Abhay Thottassery
AU - Jorden Smith
AU - Huda Asif
AU - Brian Soto
AU - Christian Arce Guzman
AU - Jalen Hammonds
AU - Anna Papasarantou
AU - Agamemnon Bakakos
AU - Ourania Papaioannou
AU - Apostolos Matsioulas
AU - Foteini Karakontaki
AU - Konstantinos Loverdos
AU - Eirini Kefalidou
AU - Brenda M Juan-Guardela
AU - Jose D Herazo-Maya
AU - Adriano R Tonelli
AU - Debabrata Bandyopadhyay
PY - 2026
DA - 2026/10/05
JO - Frontiers in medicine
DO - 10.3389/fmed.2026.1908302
UR - https://europepmc.org/article/MED/42898971
AB - Background Pulmonary hypertension (PH) is a common comorbidity of interstitial lung disease (ILD). Timely detection may improve outcomes through implementation of PH specific treatments.Methods Retrospective, multicenter study aiming to identify features that predict PH in patients with ILD. In the derivation cohort (ILD cohort-University of South Florida), we identified predictors of PH in ILD and created a scoring system using the rounded value of odds ratio for each significant parameter. Correlation with hemodynamic parameters was investigated in the derivation and validation cohort (Idiopathic Pulmonary Fibrosis cohort-Cleveland Clinic).Results We included 164 patients with ILD that underwent right heart catheterization (RHC) [derivation cohort: n = 118, median age (years, 95% CI): 69.0 (65.9-71.0), female gender: 65 (54.6%), median mean pulmonary arterial pressure (mPAP) (mmHg, 95% CI): 25.0 (23.0-27.0), median pulmonary vascular resistance (PVR) [Wood units (WU), 95% CI]: 3.6 (3.0-4.3), validation cohort: n = 46, median age (95% CI): 63.0 (60.0-66.0), female gender: 17 (37%), median mPAP (mmHg, 95% CI): 20.5 (18.0-27.2), median PVR (WU, 95% CI): 3.1 (2.2-3.4)]. Parameters and optimal cut-offs that significantly predicted precapillary PH in the derivation cohort were: age>42 years, heart rate recovery (HRR) at 1 min ≤ 15 bpm in 6-min walk test (6 MWT), 6-min walk distance (6 MWD) ≤ 270 meters, B-type natriuretic peptide (BNP) > 136 pg/mL, pulmonary artery to ascending aorta diameter ratio (PA/AA) > 1 in high-resolution computed tomography (HRCT), main PA diameter > 29 mm in HRCT, right ventricular systolic pressure > 42 mmHg on echocardiogram. We created a PH detection model that ranged from 0 to 28. The optimal cut-off threshold for the detection of PH was > 14 (80% sensitivity, 68% specificity, AUC: 0.78). A simplified scoring system without BNP and main PA diameter (range: 0-21) maintained the same AUC. The simplified scoring system exhibited positive correlation with mean pulmonary artery pressure both in the derivation (rho = 0.47, p < 0.0001) and validation cohort (rho = 0.36, p = 0.013).Conclusion This study demonstrates that incorporation of demographic, functional, imaging, and echocardiographic characteristics can optimize the early identification of precapillary PH in patients with ILD, paving the way to structured screening strategies. Timely detection of PH might lead to timely implementation of emerging PH specific treatments in patients with ILDs.
SN - 2296-858X
ER -