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Review
. 2024 Jan 10;14(1):e12310.
doi: 10.1002/pul2.12310. eCollection 2024 Jan.

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

Oksana A Shlobin  1 , Eric Shen  2 , Stephen J Wort  3 , Lucilla Piccari  4 , John A Scandurra  5 , Paul M Hassoun  6 , Sylvia M Nikkho  7 , Steven D Nathan  1
Affiliations
Review

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

Oksana A Shlobin et al. Pulm Circ. .

Abstract

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.

Keywords: idiopathic pulmonary fibrosis; management; parenchymal lung disease; pulmonary vascular disease.

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Conflict of interest statement

Dr. Oksana A. Shlobin has consulted for United Therapeutics, Bayer, Altavant, Aerovate, Jenssen&Jenssen and Merck, and is on the speaker bureau for UT, Bayer, and J&J. Mr. Eric Shen is an employee of United Therapeutics Corporation and owns stocks/shares in the company. Dr. Lucilla Piccari has received research funding from and served as a speaker for Janssen and Ferrer, advised Janssen, Ferrer, and United Therapeutics as well as received support for attending congresses from Janssen, MSD, and Ferrer, all of which not related to this manuscript. Dr. John A. Scandurra is an employee of Aria CV Inc. and owns stocks/shares in the company. Dr. Paul M. Hassoun serves on a scientific advisory board for Merck, an activity unrelated to the current work. Dr. Stephen J. Wort received honoraria from Janssen, MSD, Bayer, and Acceleron for advisory boards, received honoraria from Janssen for educational activity, received unrestricted research grants from Janssen and Bayer, and travel grants, conference registration and accommodation from Actelion and GSK. Dr. Sylvia M. Nikkho is an employee of Bayer AG. Dr. Steven D. Nathan a consultant for United Therapeutics, Bellerophon, Third Pole, Roche, Boehringer‐Ingelheim, Merck, and Daewoong.

Figures

Figure 1
Figure 1
Pulmonary hypertension (PH) in interstitial lung disease (ILD) treatment according to disease phenotype. Patients with ILD and PH might present different phenotypes according to the severity of both ILD and PH. These phenotypes sit across a spectrum for both conditions; the phenotype should be taken into account when therapeutic strategies are considered for PH‐ILD patients.
Figure 2
Figure 2
Holistic management of PH in ILD. When treating patients with ILD associated with PH it is recommended to take an holistic approach: in addition to considering pulmonary vasodilator drugs, several interventions such as lung transplant referral, optimal oxygen and antifibrotic therapy, the correct treatment for comorbidities, dyspnea and palliative care management, pulmonary rehabilitation and finally enrollment in available clinical trials may help improve the outcomes of these patients. ILD, interstitial lung disease; PDE5‐i, phosphodiesterase‐5 inhibitors; PH, pulmonary hypertension; PVD, pulmonary vascular disease.
See this image and copyright information in PMC

References

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