Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system.

Background

Interstitial lung disease associated with genetic disorders of the surfactant system is a rare entity in adults that can lead to lung transplantation. Our objective was to describe the outcome of these patients after lung transplantation.

Methods

We conducted a retrospective, multicentre study, on adults who underwent lung transplantation for such disease in the French lung transplant centres network, from 1997 to 2018.

Results

20 patients carrying mutations in

Conclusions

Lung transplantation in adults with interstitial lung disease associated with genetic disorders of surfactant system may be a valid therapeutic option. Our data suggest that these patients may have a good prognosis. Immunosuppressive protocol was not changed for these patients, and close lung cancer screening is needed before and after transplantation.

Informations de copyright

Copyright ©The authors 2023.

Déclaration de conflit d'intérêts

Conflict of interest: V. Cottin reports grants, personal fees and nonfinancial support from Boehringer Ingelheim, personal fees and nonfinancial support from Roche/Promedior, and personal fees from Celgene/BMS, Galapagos, Galecto, Shionogi, Fibrogen, RedX and PureTech, outside the submitted work. Conflict of interest: R. Borie reports grants or contracts from Roche and Boehringer Ingelheim, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Roche, Boehringer Ingelheim and Sanofi, outside the submitted work; and participation on a Data Safety Monitoring Board or Advisory Board for Savara, outside the submitted work. Conflict of interest: P-A. Thomas reports consulting fees from AstraZeneca, outside the submitted work; payment for expert testimony from Ethicon Endosurgery, outside the submitted work; and support for attending meetings and/or travel from Europrisme, outside the submitted work. Conflict of interest: S. Hirschi reports receiving a research grant from Agence Biomedecine and CSL Behring, outside the submitted work; and payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Roche and Boehringer, outside the submitted work. Conflict of interest: The remaining authors have nothing to disclose.