Affiliation:
1. Advanced Lung Disease and Transplant Program Inova Health System Falls Church Virginia USA
2. United Therapeutics Corporation Research Triangle Park North Carolina USA
3. National Heart and Lung Institute Imperial College London London UK
4. Department of Pulmonary Medicine Hospital del Mar Barcelona Spain
5. Aria CV Inc. St. Paul Minnesota USA
6. Department of Medicine, Division of Pulmonary and Critical Care Medicine Johns Hopkins University Baltimore Maryland USA
7. Global Clinical Development Bayer AG Berlin Germany
Abstract
AbstractPulmonary hypertension (PH) due to interstitial lung disease (ILD), a commonly encountered complication of fibrotic ILDs, is associated with significant morbidity and mortality. Until recently, the studies of pulmonary vasodilator therapy in PH‐ILD have been largely disappointing, with some even demonstrating the potential for harm. This paper is part of a series of Consensus Statements from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative for Group 3 Pulmonary Hypertension, with prior publications covering pathogenesis, prevalence, clinical features, phenotyping, clinical trials, and impact of PH‐ILD. It offers a comprehensive review of and a holistic approach to treatment of PH‐ILD, including the management of underlying interstitial lung diseases, importance of treating the comorbidities, emphasis on importance of exercise and palliation of dyspnea, and review of the most up‐to‐date guidelines for referral for potential lung transplant work up. It also summarizes the prior, ongoing, and possibly future studies in treatment of the vascular derangement of this morbid condition.
Subject
Pulmonary and Respiratory Medicine