Sitbon, Olivier https://orcid.org/0000-0002-1942-1951
Sahay, Sandeep https://orcid.org/0000-0002-0672-1680
Escribano Subías, Pilar https://orcid.org/0000-0002-6640-4839
Zolty, Ronald L. https://orcid.org/0000-0003-1749-4046
Kingrey, John F. https://orcid.org/0000-0003-3166-2069
Ryan, John J.
Sobol, Irina https://orcid.org/0000-0002-1328-3447
Sood, Namita
Benza, Raymond L. https://orcid.org/0000-0001-9627-6236
Channick, Richard N. https://orcid.org/0000-0002-9016-4468
Chin, Kelly M. https://orcid.org/0000-0002-1214-6723
Frantz, Robert P. https://orcid.org/0000-0003-4128-3978
Ghofrani, Hossein-Ardeschir https://orcid.org/0000-0002-2029-4419
Hemnes, Anna R. https://orcid.org/0000-0002-2755-5845
McLaughlin, Vallerie V. https://orcid.org/0000-0002-8907-1651
Vachiéry, Jean-Luc https://orcid.org/0000-0002-6358-7852
Zamanian, Roham T. https://orcid.org/0000-0002-0734-0994
ter Veer, Anna
Roscigno, Robert F.
Mottola, David
Parsley, Ed
Aranda, Richard
Zisman, Lawrence S.
Howard, Luke S. https://orcid.org/0000-0003-2822-210X
,
Funding for this research was provided by:
GB002
Université Paris-Saclay
Article History
Received: 17 April 2025
Accepted: 26 June 2025
First Online: 11 August 2025
Declarations
:
: Olivier Sitbon reports having received grants or contracts for research and education (payment made to the institution) from Janssen, MSD, and Ferrer; received grants or contracts for education (payment made to the institution) from AOP Orphan; payment or honoraria for lectures from AOP Orphan and Ferrer; and for educational events, lecturer, from Janssen and MSD; support for attending meetings or travel (travel costs) from MSD and Janssen; and participated on an Advisory Committee for Gossamer Bio, Inc., AOP Orphan, Enzyvant, Ferrer, Janssen, Liquidia, Roivant, MSD, and United Therapeutics. Sandeep Sahay reports having received consulting fees from Janssen, United Therapeutics, Liquidia, Keros, and Merck; support for attending meetings and/or travel from Janssen for presenting scientific work at WSPH meeting; patents submitted for Janssen as inventor, secondary titration of Uptravi; participation on an advisory board for United Therapeutics, Bayer, Liquidia, Merck, and Gossamer Bio, Inc.; participation as Chair and member of a Data Safety Monitoring Board for NIH-funded trials; holding a leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid as PVD section Chair, ACCP, CHEST, Member of scientific leadership committee PHA. Pilar Escribano Subías reports having received payment or honoraria for lectures, presentations, speaker bureaus, manuscript writing, or educational events from Janssen, MSD, Ferrer, AOT, and Aerovate; support for attending meetings and/or travel from Janssen and MSD; participation on a data safety monitoring board or advisory board for Gossamer Bio, Inc., Janssen, MSD, AOT, Aerovate, and Ferrer; and receipt of equipment, materials, drugs, medical writing, gifts, or other services from Gossamer Bio, Inc. Ronald L. Zolty reports having received consulting fees from Johnson & Johnson, United Therapeutics, Bayer, and Alnylam. John J. Ryan reports having received payment or honoraria for lectures, presentations, speaker bureaus, manuscript writing or educational events from Bayer, Merck, Janssen PH, United Therapeutics, and Liquidia. Namita Sood reports having received consulting fees from United Therapeutics and Merck; membership in a Speakers Bureau for Bayer and BI; and membership in an Advisory Board for United Therapeutics and Merck. Raymond L. Benza reports having received consulting fees from Merck, United Therapeutics, KEROS, Aerovate, Insmed, and Cereno; served as an advisory committee member for Gossamer Bio, Inc.; served as an advisory board member for Altavant, Merck, Janssen, and Insmed; served on a data safety monitoring board for Janssen; and served on a scientific advisory board for Cereno. Richard N. Channick reports having received consulting fees from Gossamer Bio, Inc., Janssen, Bayer, United Therapeutics, and Third Pole; payment or honoraria for lectures from Janssen and Bayer; and participated on an advisory committee for Gossamer Bio, Inc., Janssen, and Bayer. Kelly M. Chin reports having received grants or contracts (fees to institution) for clinical studies overseen by her from Gossamer Bio, Inc., Janssen, United Therapeutics, Merck, and Altavant; received fees for work on an advisory board for Merck; received fees for work as an advisory committee member for Gossamer Bio, Inc.; received fees for work as a steering committee member for Janssen; received fees for work on an adjudication committee for United Therapeutics; and received fees for an editorial role with the American Heart Association. Robert P. Frantz reports having royalties or licenses from UpToDate; receiving consulting fees from Janssen and Liquidia; participated on a Data Safety Monitoring board for Aerovate; participated as an Advisory Committee member for Gossamer Bio, Inc.; participated on an Advisory Board for Janssen, Liquidia, Tenax Therapeutics, ShouTi, Insmed, and Merck. Hossein-Ardeschir Ghofrani reports having received consulting fees from Gossamer Bio, Inc., Aerovate, Altavant, Bayer AG, Attgeno, Janssen/Actelion, MSD/Acceleron, and Pfizer; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events as a speaker from Bayer AG, Janssen/Actelion, and MSD/Acceleron; participated as an Advisory Committee member for Aerovate, Altavant, Bayer AG, Attgeno, Janssen/Acctelion, MSD/Acceleron, and Pfizer; and as a member of a Data and Safety Monitoring Board for Insmed. Anna R. Hemnes reports having received grants or contracts from NHLBI; consulting fees from United Therapeutics, Tenax Therapeutics, Merck, and Janssen; having served as an Advisory Committee member for Gossamer Bio, Inc.; and having held stock from Tenax Therapeutics. Vallerie V. McLaughlin reports having received grant support from Aerovate, Enzyvant/Altavant, Gossamer Bio, Inc., Janssen, Merck/Acceleron, Sonovie, and Keros; consulting fees from Aerami, Aerovate, Altavant, Apollo, Bayer, CVS/Caremark, LLC, Corvista, Gossamer Bio, Inc., Janssen, Keros, Liquidia, Merck, Morphic, Regeneron, Respira, Roivant, United Therapeutics, and Vertex; and participated as an advisory committee member for Gossamer Bio, Inc. Jean-Luc Vachiéry reports having received consulting fees from Gossamer Bio, Inc., Insmed, and Tectonic; and consulting fees paid to his institution from Aerovate, Janssen, Enzyvant, Bayer HealthCare, Merck, Liquidia, and Aerami; received payment or honoraria (payment to institution) for lectures, presentations, speakers bureaus, manuscript writing or educational events from Janssen, Merck, Novartis, and Boehringer Ingelheim; received payment for expert testimony from Actelion Pharmaceuticals; received either support for attending meetings or travel (payment to institution) from Merck; participated on a data safety monitoring board (payment to institution) from Janssen, GSK, and Moderna; membership on Steering Committees for Gossamer Bio, Inc., Insmed, Merck, and United Therapeutics. Roham T. Zamanian reports having received grants or contracts for industry-supported research from United Therapeutics, Janssen, Tenax Therapeutics, and Gossamer Bio, Inc.; consulting fees from Janssen, Vivus, Morphogen-IX, Merck, and Gossamer Bio, Inc.; and stock or stock options from Selten. Luke S. Howard reports having received payment or honoraria for speaker bureau membership from Janssen; participated as an Advisory Board member for Janssen, MSD, Altavant, United Therapeutics, Keros Therapeutics, and Liquidia, and as an Advisory Committee member for Gossamer Bio, Inc.; and having stock or stock options (shareholder) at ATXA Therapeutics, iOWNA, Circular, and Calibre Biometrics. Anna ter Veer, Robert F. Roscigno, David Mottola, Ed Parsley, Richard Aranda, and Lawrence S. Zisman, report stocks or stock options from Gossamer Bio, Inc.; and employment at Gossamer Bio, Inc. Lawrence S. Zisman also reports patents related to seralutinib (assigned to Gossamer Bio, Inc., or Pulmokine) and other financial or non-financial interests (payments) from Xoma, Inc. Olivier Sitbon, Pilar Escribano Subías, Ronald L. Zolty, John F. Kingrey, John J. Ryan, Irina Sobol, Namita Sood, Raymond L. Benza, Richard N. Channick, Kelly M. Chin, Robert P. Frantz, Hossein-Ardeschir Ghofrani, Anna R. Hemnes, Vallerie V. McLaughlin, Jean-Luc Vachiéry, Roham T. Zamanian, and Luke S. Howard, report receiving medical writing and editing support, and rapid service fee support, for the present manuscript funded by Gossamer Bio, Inc. and Open Access fee support from the Université Paris-Saclay.
: The OLE protocol and all study-related documents were approved at each study center by the institutional review board or independent ethics committee for each site (Supplementary material). The OLE study was conducted in accordance with the tenets of the Declaration of Helsinki and the International Conference on Harmonisation-Good Clinical Practice, and all applicable laws and regulations. Prior to participation, all patients provided written informed consent.