Document is current

Article History

Accepted: 11 December 2025

First Online: 12 January 2026

Declarations

:

: Pilar Escribano-Subias has received grants/research support from Ferrer; consulting fees from Johnson & Johnson, Ferrer and MSD; payment or honoraria for lectures, presentations, speaker’s bureaus, manuscript writing or educational events from Johnson & Johnson, MSD, Ferrer and AOT; has received support from Johnson & Johnson and MSD for attending meetings and/or travel; and has participated on a Data Safety Monitoring Board or Advisory Board for Johnson & Johnson, MSD, Ferrer, Gossamer, AOT, and Aerovate. Murali M. Chakinala has received research support from Johnson & Johnson, Bayer, Medtronic, United Therapeutics, Merck, Gossamer Bio and Respira, and has served on consulting/advisory boards for Johnson & Johnson, Aerovate, Liquidia, United Therapeutics, Merck, Tectonic Therapeutics and Bayer. Kelly Chin has served as a scientific committee member for Johnson & Johnson; has received research grants to institution from Johnson & Johnson, Gossamer Bio, Merck and United Therapeutics; has received support for travel to meetings from Johnson & Johnson; and has received consultancy fees from Johnson & Johnson, Gossamer Bio, Merck and United Therapeutics. Eva-Maria Didden is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Audrey Muller is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Tatiana Remenova is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Apoorva Venkatesh is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Mohammad Rahman is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Gurinderpal Doad is an employee of Johnson & Johnson and owns shares of stock/stock options in Johnson & Johnson. Harrison W. Farber is/was a consultant for Aerami, Aerovate, Johnson & Johnson, United Therapeutics, Acceleron (Merck), Altavant and Pulmovant, and is a Speaker for Bayer (non-branded). Sean Gaine has had relations, such as funding, with the following subjects that have commercial interests in the pharmaceutical and medical field: Aerovate Therapeutics, Acceleron Pharma Inc, Altavant, AOP, Gossamer Bio, Johnson & Johnson, MSD and United Therapeutics. Anna R. Hemnes has served as a consultant for Merck, Gossamer Bio, United Therapeutics, Johnson & Johnson and Tenax Therapeutics. She is a shareholder in Tenax Therapeutics. Kristin Highland receives grants/contracts, consults and/or is on the Speaker’s Bureau for Johnson & Johnson, United Therapeutics, Gossamer Bio and Bayer healthcare. Tobias Lange has received speaker fees and/or consultancy fees and/or financial and non-financial support for participation in scientific events and/or participated on a Data Safety Monitoring Board or Advisory Board for Acceleron Pharma, AstraZeneca, AOP orphan pharmaceuticals, Bayer, BMS, Böhringer Ingelheim, CGI medicare, Ferrer, Gossamer Bio, Johnson & Johnson, MSD and Pfizer. Vallerie McLaughlin has received grant support from Aerovate, Gossamer Bio, Keros and Sonovie (as a Principal Investigator) and from Johnson & Johnson and Merck/Acceleron (as a sub-Investigator), and has served as a scientific consultant for Aerovate, CVS/Caremark, L.L.C, Gossamer Bio, Johnson & Johnson, Keros, Liquidia, Merck, Morphic, Regeneron, Respira, Roivant and United Therapeutics. Stefan Söderberg has received compensation for consultancy or speaking for Johnson & Johnson and Merck. Nick H. Kim has consulted for, received research grants from, or spoken for Johnson & Johnson, Bayer, Merck, United Therapeutics, Gossamer Bio and Pulnovo.

: Although these data are not currently publicly available for sharing, requests for sharing can be sent to the Corresponding Author and will be evaluated on an individual basis.

: The SPHERE and EXPOSURE study protocols and amendments were approved by the relevant Independent Ethics Committees/Institutional Review Boards.

: Written informed consent to participate in the SPHERE and EXPOSURE studies was obtained from each patient.

: Not applicable.

: Not available.

: Pilar Escribano Subias: conceptualisation, investigation, writing—review and editing. Murali M. Chakinala: conceptualisation, investigation, writing—review and editing. Kelly M. Chin: conceptualisation, investigation, writing—review and editing. Eva-Maria Didden: conceptualisation, methodology, software, formal analysis, investigation, writing—review and editing. Gurinderpal Doad: conceptualisation, methodology, investigation, writing—review and editing. Harrison W. Farber: conceptualisation, investigation, writing—review and editing. Sean Gaine: conceptualisation, investigation, writing—review and editing. Anna R. Hemnes: conceptualisation, investigation, writing—review and editing. Kristin B. Highland: conceptualisation, investigation, writing—review and editing. Tobias J. Lange: conceptualisation, investigation, writing—review and editing. Vallerie McLaughlin: conceptualisation, investigation, writing—review and editing. Audrey Muller: conceptualisation, methodology, formal analysis, investigation, project administration, writing—review and editing, visualisation. Mohammad Rahman: conceptualisation, methodology, formal analysis, investigation, writing—review and editing. Tatiana Remenova: conceptualisation, methodology, investigation, writing—review and editing. Stefan Söderberg: conceptualisation, investigation, writing—review and editing. Apoorva Venkatesh: conceptualisation, methodology, software, formal analysis, data curation; investigation, writing—review and editing. Nick H. Kim: conceptualisation, investigation, writing—review and editing. All authors read and approved the final version.