Pulmonary hypertension associated with interstitial lung disease: a state-of-the-art review
Year: 2026
Type: article
Abstract: Pulmonary hypertension (PH) is a common sequela of interstitial lung diseases (ILDs) and is associated with poor prognosis and quality of life. The diagnosis and management of PH associated with ILD (ILD-PH) are challenging, due in part to the heterogeneity of ILD subtypes, difficulty distinguishing symptoms and signs of ILD progression from manifestations of PH, lack of specific biomarkers, and the requirement of invasive right heart catheterisation (RHC) to diagnose and assess the severity of PH. This state-of-the-art review provides a comprehensive overview of the clinical characteristics, pathophysiology, diagnosis, prognosis and treatment of ILD-PH. It also identifies promising areas for future research, such as the development and validation of novel biomarkers and imaging techniques and further evaluation of the efficacy and safety of pharmacological therapies for PH in patients with ILD. Given the inherent complexity of diagnosing and managing heterogeneous ILD subtypes, there is a clear need for multidisciplinary and personalised care strategies for ILD-PH. Dedicated attention and further research to improve diagnostic and treatment interventions are warranted to help develop much-needed, evidence-based guidelines and to improve outcomes that are meaningful for patients with ILD-PH.
Source: European Respiratory Journal
Institutions: University of Washington, Houston Methodist, Royal Brompton Hospital, Imperial College London, University of Alberta, LMU Klinikum, German Center for Lung Research, Ludwig-Maximilians-Universität München, Inserm, Université Paris-Saclay, Assistance Publique – Hôpitaux de Paris, Bicêtre Hospital, United Therapeutics (United States), University of Michigan, Cleveland Clinic, Cleveland Clinic Florida, Mayo Clinic, University of California, Los Angeles
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Funders: American Thoracic Society, Amgen, Bristol-Myers Squibb, Pfizer, AstraZeneca, Sanofi, United Therapeutics Corporation, European Respiratory Society, Regeneron Pharmaceuticals, Acceleron, Houston Methodist Hospital, Moderna, Insmed, University of Cambridge, AOP Orphan, National Institutes of Health, Houston Methodist Research Institute, Janssen Pharmaceuticals
OpenAlex ID: W7135125508