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Review
. 2022 Jul 1;12(3):e12127.
doi: 10.1002/pul2.12127. eCollection 2022 Jul.

Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative-Group 3 pulmonary hypertension

Sylvia M Nikkho  1 , Manuel J Richter  2 , Eric Shen  3 , Steven H Abman  4 , Katerina Antoniou  5 , Jonathan Chung  6 , Peter Fernandes  7 , Paul Hassoun  8 , Howard M Lazarus  9 , Horst Olschewski  10 , Lucilla Piccari  11 , Mitchell Psotka  12   13 , Rajan Saggar  14 , Oksana A Shlobin  15 , Norman Stockbridge  16 , Patrizio Vitulo  17 , Carmine Dario Vizza  18 , Stephen J Wort  19   20 , Steven D Nathan  15
Affiliations
Review

Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative-Group 3 pulmonary hypertension

Sylvia M Nikkho et al. Pulm Circ. .

Abstract

Pulmonary hypertension (PH) has been linked to worse outcomes in chronic lung diseases. The presence of PH in the setting of underlying Interstitial Lung Disease (ILD) is strongly associated with decreased exercise and functional capacity, an increased risk of hospitalizations and death. Examining the scope of this issue and its impact on patients is the first step in trying to define a roadmap to facilitate and encourage future research in this area. The aim of our working group is to strengthen the communities understanding of PH due to lung diseases and to improve the care and quality of life of affected patients. This introductory statement provides a broad overview and lays the foundation for further in-depth papers on specific topics pertaining to PH-ILD.

Keywords: epidemiology; interstitial lung disease; prognosis; pulmonary hypertension; symptom assessment and management.

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Conflict of interest statement

Dr. Nikkho is an employee of Bayer AG; Dr. Richter received funding from the JLU‐CAREER program (German Research Foundation, DFG, 413584448) and from the Collaborative Research Center (SFB) 1213—Pulmonary Hypertension and Cor Pulmonale, grant number SFB1213/1, project B08 (German Research Foundation, Bonn, Germany); Eric Shen is an employee of United Therapeutics Corporation; Dr. Abman serves as a scientific advisor to Oak Hill Bio and NHLBI grants HL68702, HL145679 and UHL151458 not related to this manuscript; Dr. Antoniou has no conflict of interest.; Dr. Chung has no conflict of interest as it pertains to this paper; Peter Fernandes is an employee of Bellerophon Pharma; Dr. Hassoun serves on a scientific advisory board for Merck, an activity unrelated to the current work; Howard M. Lazarus is an employee of Altavant Sciences; Dr. Olschewski received funding from Actelion, Algorithm Sciences, AOP, Astra Zeneca, Bayer, Boehringer, Chiesi, GSK, Janssen, Menarini, MSD, Novartis, Ludwig Boltzmann Society, Ferrer, MedUpdate, and Mondial not related to this work; Dr. Piccari has received research funding from and served as a speaker for Janssen as well as received support for attending congresses from Janssen, MSD and Ferrer, not related to this manuscript; Dr. Psotka has no conflict of interest; Dr. Saggar has a Consulting and Advisory Role for United Therapeutics, Third Pole, Novartis, Acceleron, Aerovate, and Janssen; Dr. Shlobin has consulted for UT, Bayer, ALtavant, Aerovate, Jenssen & Jenssen, and Merck, and is on the speaker bureau for UT, Bayer, and J&J; Dr. Stockbridge has no conflict of interest; Dr. Vizza has no conflict of interest regarding this topic; Dr. Vitulo has no conflicts of interest to disclose; Dr. S. John 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. Nathan is a consultant for United Therapeutics, Bellerophon, Third Pole, Roche, Boehringer‐Ingelheim, Merck and Daewoong.

Figures

Figure 1
Figure 1
Continuum of pulmonary vasculopathy in interstitial lung disease in the context of histologic abnormalities and serial echocardiographic changes.
Figure 2
Figure 2
Concept depiction of the overlapping symptom burden of ILD and complicating PH. The x axis depicts the progressive nature of the symptoms over time, while the y axis depicts the magnitude of the global symptom burden imposed by the ILD and PH. The dashed symptoms are less common and do not occur in all patients. ILD, interstitial lung disease; PH, pulmonary hypertension.
See this image and copyright information in PMC

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