Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative—Group 3 Pulmonary Hypertension

作者：Oksana A. Shlobin, Eric Shen, Stephen John Wort, Lucilla Piccari, John Scandurra, Paul M. Hassoun, Sylvia M. Nikkho, Steven D. Nathan · 发表于：Pulmonary Circulation · 年份：2024 · DOI：10.1002/pul2.12310 · 被引用次数：23 · 研究领域：Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Pulmonary Hypertension Research and Treatments、Medical Imaging and Pathology Studies

Pulmonary 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.