Elevated mean pulmonary artery pressure and idiopathic pulmonary fibrosis (IPF) were associated with increased mortality risk among individuals with interstitial lung disease (ILD) and pulmonary hypertension (PH), based on new interim data from the PHINDER study presented at the at American College of Chest Physicians (CHEST) 2026 Annual Meeting.

Although patients with ILD and PH experience significant morbidity and mortality, PH screening guidance for patients with ILD is lacking, and PH often goes unidentified and undiagnosed, noted Parth M. Rali, MD, director of the Temple University Health System Pulmonary Embolism Response Team Program, Section Temple Lung Pulmonary Vascular Disease Program, Philadelphia, and colleagues.

Treatment for patients with PH-ILD has become more feasible in the wake of the FDA’s 2025 approval of treprostinil, which has been shown to help patients with both conditions, said Rali. Studies to examine the mechanism of action are underway, but a tool to stratify patients was lacking, he added.

In response to a request for collaboration with United Therapeutics (but without dedicated funding from the company), Rali and colleagues at Temple University developed a diagnostic score for patients with ILD and PH to better predict outcomes and inform treatment.

The original PHINDER study, a prospective, noninterventional study, was designed to identify screening strategies for PH in patients with ILD. In their poster, Rali and colleagues used a validated scoring model known as PVD-B65 to predict 1-year mortality in 119 study enrollees with ILD and confirmed PH. The model’s components include pulmonary fibrosis without emphysema, pulmonary vascular resistance greater than 5 Wood Units, less than 150 m on the 6-minute walk test, and B-type natriuretic peptide greater than 200 pg/dL. Scores are based on a point system and categorized as low risk (0-3 points), intermediate risk (4-10 points), or high-risk (11-14 points) for mortality at 1 year.

The most common ILD subtype was IPF, in 41% of the patients, followed by connective tissue disease-associated ILD (23%), nonspecific interstitial pneumonia (17%), chronic hypersensitivity pneumonitis (9%), and other subtypes (10%). Patients also were stratified by mean pulmonary arterial pressure (mPAP) elevation as mild (21-24 mm Hg) or moderate-severe (≥ 25 mm Hg).

Overall, the strongest predictors of mortality in patients with ILD and PH were IPF and elevated mPAP scores. Among patients with mild vs moderate-severe mPAP elevation, the mean PVD-B65 risk scores were 4.2 and 5.3, respectively. When the patients were further stratified by ILD subtype, the mean PVD-B65 risk score was highest (7.9) among the 49 patients with IPF.

Additionally, 6% of patients with moderate-severe mPAP and 12% of those with a diagnosis of IPF met the high-risk criteria using the PVD-B65.

The findings were limited by several factors including the relatively small sample size, but the results support the need for earlier PH screening in ILD, especially among individuals with IPF, the researchers noted.

However, the new data represent a first attempt to see how the patients would land in stratification after applying the score, Rali explained.

Ultimately, final results of the PHINDER study will be used to develop a validated tool for earlier detection of PH in patients with ILD. “Ideally, applying the score can help clinicians understand the full journey of these patients,” said Rali.

Meeting the Need for Better Screening

“Identifying which patients are at the highest risk of mortality may help clinicians to identify individuals who warrant more thorough screening, closer monitoring, and timely consideration of treatment,” said Ariel McKenna, DO, a pulmonary critical care physician and PH specialist at Tufts Medical Center in Boston. The new study is especially relevant because of the limited diagnostic accuracy of echocardiography in patients with chronic lung disease, said McKenna, who was not involved in the study.

“By identifying predictors of adverse outcomes in this population, this study may help in the development of more effective screening to ultimately support earlier recognition and improved management of PH in patients with ILD,” she said.

The finding that IPF was the ILD subtype associated with the highest mortality risk was not surprising, as IPF is generally associated with a poor prognosis and often has more limited treatment options, said McKenna. Overall, the study findings reaffirm the importance of recognizing PH in patients with ILD and highlight the need for effective screening strategies and earlier diagnosis to facilitate risk stratification and management, she said. Ideally, the results will encourage more screening and evaluation for PH in clinical practice, she added.

However, further research is needed not only to better characterize the clinical predictors associated with the development of PH in patients with ILD, but also to explore additional treatment options, McKenna noted. “Ultimately, identifying ways to reduce mortality in this population would be ideal,” she said.

The original PHINDER study is sponsored by United Therapeutics. Rali and Mckenna disclosed having no financial conflicts of interest.