Pulmonary hypertension (PH) increases the morbidity and mortality risk in interstitial lung disease (ILD), but evidence-based guidance for detecting PH, defined as a mean pulmonary arterial pressure (mPAP) > 20 mmHg, remains limited in this population.¹ The prospective PHINDER study put adults with ILD through routine noninvasive testing followed by right heart catheterization, with the goal of building an evidence-based PH screening tool.¹ An exploratory interim analysis presented at the CHEST 2026 Annual Meeting in Phoenix applied the validated PVD-B65 model, which predicts 1-year mortality, to 119 patients with PH.¹

The score assigns points for fibrotic ILD, BNP > 200 pg/mL, age > 65 years, pulmonary vascular resistance > 5 WU, and 6-minute walk distance < 150 m.¹,² Patients with mPAP of ≥ 25 mmHg had a numerically higher mean score than those with mPAP of 21 to 24 mmHg (5.3 ± 3.2 vs 4.2 ± 2.8), and fewer were low risk (38% vs 60%).¹ Idiopathic pulmonary fibrosis (IPF) had the highest mean score (7.9 ± 1.9), and no patients with IPF were low risk, versus 64% with hypersensitivity pneumonitis, 78% with connective tissue disease, and 80% with nonspecific interstitial pneumonia.¹

The investigators concluded the findings support earlier PH screening and timely hemodynamic assessment in patients with ILD, particularly those with IPF.¹ In the following Q&A, lead investigator Parth Rali, MD, associate professor of thoracic medicine and surgery at the Lewis Katz School of Medicine at Temple University, discusses why PH in ILD is so often detected late, how to interpret the IPF findings, and what the data should mean for screening and referral.

Q&A: Earlier Pulmonary Hypertension Screening in ILD

HCPLive: Why is PH in ILD so often caught late, and what is PHINDER trying to fix?

Parth Rali, MD: Pulmonary hypertension in ILD is often caught late because there is no structured screening pathway for those patients. The traditional pathway we use for pulmonary hypertension screening, like echocardiogram and BNP markers, may not work perfectly well in patients with advanced interstitial lung disease.

A simple example I would give is that a lot of patients with interstitial lung disease may not get a good echocardiogram window, and a negative echocardiogram does not rule out pulmonary hypertension in this group. PHINDER is trying to fix that problem by identifying, and potentially developing, a risk score tool that can be used to identify patients with pulmonary hypertension and interstitial lung disease.

HCPLive: IPF had the highest risk scores, and no patients with IPF were low risk. How much reflects true risk versus how the score is built?

Parth Rali, MD: I think what you are referring to is the PVD-B65 score, developed by Temple Lung Center physicians, as applied to the PHINDER cohort. I would like to explain that PVD-B65 was developed by a group of physicians from the Temple Lung Pulmonary Vascular Disease Department, where our sole interest was to identify patients with pulmonary hypertension related to ILD and predict their mortality.

The score was not only developed and published but also externally validated in the PVDOMICS cohort, and a manuscript is currently online in ERJ.3 In this manuscript, what we did is take the PVD-B65 score and apply it to the patients with ILD-PH in the PHINDER cohort. Not surprisingly, we noted that patients with IPF had a higher PVD-B65 score compared with other interstitial lung diseases.

So, this is the first cut at the data where we could not only diagnose patients with pulmonary hypertension but also stratify those patients in terms of 1-year mortality based on the PVD-B65 score. I’m hoping this is the beginning of the work, and we will continue to investigate further with evaluation of PFTs and chest CT scans to identify why the IPF cohort had higher PVD-B65 scores compared with other interstitial lung diseases.

HCPLive: Even mildly elevated pressures carried meaningful risk. What should that mean for screening and referral?

Parth Rali, MD: Yes, any elevation of pulmonary hypertension in a patient with ILD carries significant mortality and morbidity risk for those patients. All of these patients with ILD-PH absolutely should be referred to the nearest lung transplant center.

Looking at the PHINDER cohort and our PVD-B65 application, I think it is up to clinicians not to settle on echocardiogram and clinical judgment alone to see who has PH versus who does not. Clinicians should refer the patient for right heart [catheterization] more aggressively, more effectively, and more rapidly. [That] way, if PH exists, [the] patient can either be treated with FDA-approved medications or be enrolled in clinical trials and simultaneously be referred for [a] lung transplant.

References

1. Rali P, Dersarkissian M, Gayen S, et al. Risk stratification in interstitial lung disease associated with pulmonary hypertension using the PVD-B65 model: interim analysis from the PHINDER study. Presented at: CHEST 2026 Annual Meeting; October 18-21, 2026; Phoenix, AZ.

2. Gayen S, Pescatore J, Bittner M, et al. Development of the pulmonary fibrosis, pulmonary vascular resistance, six minute walk distance, B-type natriuretic peptide, age (PVD-B65) risk score for patients with chronic lung disease and pulmonary hypertension. BMC Pulm Med. 2025;25(1):69. doi:10.1186/s12890-025-03538-8

3. Gayden S, Pescatore J, Illipparambil L, et al. The PVD-B65 risk score for interstitial lung disease and pulmonary hypertension: External validation using the PVDOMICS cohort. ERJ Open Research. 00307-2026; DOI: https://doi.org/10.1183/23120541.00307-2026