May 02, 2026
Physicians caring for patients with advanced lung disease are frequently reminded how devastating pulmonary hypertension can be when it complicates interstitial lung disease. Pulmonary hypertension associated with interstitial lung disease (PH-ILD) significantly worsens symptoms, limits functional capacity and carries a poor prognosis. Yet, despite its clinical importance, evidence guiding treatment decisions has historically been limited.
Mayo Clinic recently contributed to an international collaboration that sought to address this knowledge gap. In a recent study published in the American Journal of Respiratory and Critical Care Medicine, investigators from the Pulmonary Vascular Research Institute (PVRI) GoDeep consortium analyzed outcomes in patients with PH-ILD across a large global network of expert centers.
The GoDeep meta-registry represents the most comprehensive international datasets in pulmonary vascular disease. "By integrating information from multiple specialized centers, the registry allows investigators to examine clinical patterns and outcomes in patient populations that are often difficult to study in traditional clinical trials," says Hector R. Cajigas, M.D., a pulmonologist and critical care specialist at Mayo Clinic in Rochester, Minnesota. In this analysis, more than 34,000 patients with pulmonary hypertension were screened. And 940 individuals with hemodynamically confirmed PH-ILD were identified for detailed evaluation.
"Mayo Clinic's participation in this effort reflects the ongoing commitment to advancing the understanding of pulmonary vascular disease through collaborative research," Dr. Cajigas affirms. As a referral center for complex pulmonary hypertension and advanced lung disease, Mayo Clinic's pulmonary, critical care and sleep medicine practice is uniquely positioned to contribute to — and learn from — large international initiatives such as GoDeep.
One of the most striking observations from this study was the severity of the prognosis associated with PH-ILD. Despite advances in the treatment of pulmonary hypertension in other disease groups, patients with PH-ILD continue to face substantial mortality risk. In this cohort, overall survival was about 35% at three years and 22% at five years after diagnosis. These sobering numbers highlight the urgent need for improved strategies to identify high-risk patients and intervene earlier.
A key insight from the analysis was the central role of pulmonary vascular resistance (PVR) in determining outcomes. Patients with more-severe pulmonary vascular disease — defined by a PVR greater than 5 Wood units — had significantly worse survival compared with those with milder hemodynamic abnormalities.
"This finding reinforces the importance of careful hemodynamic evaluation using right heart catheterization, particularly in patients whose symptoms seem disproportionate to the severity of their underlying lung disease," Dr. Cajigas observes.
The study also provided important insights into real-world treatment practices across expert centers. More than half of the patients in the registry received pulmonary hypertension-targeted therapy, most commonly phosphodiesterase-5 (PDE) inhibitors such as sildenafil. Although these medications are widely used in clinical practice, evidence supporting their role in PH-ILD has remained limited. There is no prospective data that supports use of PDE inhibitors for PH-ILD.
Encouragingly, the analysis found that treatment with PDE5 inhibitors was associated with improved survival in patients with severe PH-ILD, particularly those with idiopathic interstitial pneumonia and idiopathic pulmonary fibrosis.
Importantly, this association was not observed in patients with less severe pulmonary vascular disease, suggesting that careful patient selection may be critical when considering targeted therapies.
The potential benefit of combination therapy also was important. Patients receiving both PDE5 inhibitors and inhaled prostacyclin analogues appeared to have better outcomes than those treated with PDE5 inhibitors alone.
While these results should be interpreted cautiously given the observational nature of registry data, they provide an important signal that combination strategies may warrant further investigation in future clinical trials.
Participating in this global collaboration underscores the value of specialized referral centers working together to generate meaningful evidence. Registries such as GoDeep allow clinicians and investigators to study rare and complex conditions at a scale that would otherwise be impossible.
Equally important, studies like this help inform conversations with patients and referring physicians. They highlight the importance of early recognition of pulmonary hypertension in patients with interstitial lung disease and reinforce the role of referral to experienced pulmonary hypertension centers for comprehensive evaluation.
"The findings from this analysis provide a framework for future prospective trials aimed at improving outcomes in PH-ILD," Dr. Cajigas says. "They also reinforce the importance of multidisciplinary care — bringing together expertise in pulmonary fibrosis, pulmonary hypertension and advanced cardiopulmonary physiology — to deliver the best possible care for these patients."
Mayo Clinic remains committed to advancing both research and clinical care in pulmonary vascular disease. Through continued collaboration with international partners and dedication to innovation in patient care, Mayo Clinic's pulmonary experts hope to improve the outlook for patients facing the complex challenges of pulmonary hypertension in interstitial lung disease.
For more information
Hemodynamics and phosphodiesterase-5 inhibitor treatment associated with survival in pulmonary hypertension in interstitial lung disease: A PVRI GoDeep meta-registry analysis. American Journal of Respiratory and Critical Care Medicine. 2025;211:1855.
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