Is this patient's pulmonary hypertension more than fibrosis?

Enter the lung function of a patient with idiopathic pulmonary fibrosis. LuPNet places them among 260 simulated cases simulated with and without a structural vasculopathy, and estimates how much of their expected pulmonary artery pressure fibrosis alone would explain. For research use only; it does not replace right heart catheterization.

FVC (% predicted) DLCO (% predicted, haemoglobin-corrected) Resting SpO₂ (%, optional)

Probability of mPAP ≥ 25 mmHg

Probability of the vascular phenotype

mPAP expected from fibrosis alone

DLCO expected for this FVC without vasculopathy

25 most similar simulated cases: mPAP

… of whom with mPAP ≥ 25 mmHg

Where the patient sits

Each dot is a simulated case, coloured by its simulated mean pulmonary artery pressure. The line is the DLCO that tissue loss alone produces at each FVC; patients well below it carry the vascular phenotype.

mPAP 1535+ mmHg — expected DLCO without vasculopathy ○ this patient

How the estimates are made

The simulated cases come from LuPNet, a network model of the fibrotic lung with a shared shear-stress law for the pulmonary vessels. Each simulated case is an independent run of the model, not a real patient. Half of the cases carry a structural small-artery vasculopathy with its own course, and fibrosis progresses at a different speed in each case.
The vasculopathy was calibrated so that the cohort reproduces the distribution of mPAP in mild-to-moderate IPF (ARTEMIS-IPF), the weak correlation of mPAP with DLCO and its absence with FVC. The prevalence of PH in advanced disease was not fitted.
The probability of PH is a logistic model of FVC and FVC/DLCO fitted to the in silico cohort with realistic measurement noise (AUC 0.77). The vascular-phenotype probability comes from a two-component mixture of the DLCO deficit relative to the expected curve.
The in silico cohort spans FVC 42–80% and DLCO 7–65%. The model underestimates restriction in very advanced disease (its FVC does not fall below about 42%), so estimates at low FVC overstate the tissue contribution.