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

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 dem...

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Bibliographic Details
Published in:	Pulmonary circulation Vol. 14; no. 1; pp. e12310 - n/a
Main Authors:	Shlobin, Oksana A., Shen, Eric, Wort, Stephen J., Piccari, Lucilla, Scandurra, John A., Hassoun, Paul M., Nikkho, Sylvia M., Nathan, Steven D.
Format:	Journal Article
Language:	English
Published:	United States John Wiley & Sons, Inc 01-01-2024 John Wiley and Sons Inc Wiley
Subjects:	Drug development idiopathic pulmonary fibrosis Lung diseases management parenchymal lung disease Pulmonary hypertension pulmonary vascular disease Review United States > US parenchymal lung disease idiopathic pulmonary fibrosis pulmonary vascular disease management
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Summary:	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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 ObjectType-Review-3 content type line 23
ISSN:	2045-8940 2045-8932 2045-8940
DOI:	10.1002/pul2.12310