Pulmonary Hypertension Associated With Interstitial Lung Disease: State-of-the-Art Review

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 pharmacologic 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.