학술논문
Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system.
Document Type
Academic Journal
Author
Bermudez J; Department of Respiratory Medicine and Lung Transplantation, Assistance Publique - Hôpitaux de Marseille, Hôpital Nord; Aix-Marseille Université, Marseille, France.; Nathan N; Assistance Publique - Hôpitaux de Paris (APHP) - Sorbonne Université, Inserm Childhood Genetic Disorders and Reference Center for Rare Lung Diseases, Armand Trousseau Hospital, Paris, France.; APHP - Sorbonne Université, Pediatric Pulmonology Department and Reference Center for Rare Lung Diseases RespiRare, Armand Trousseau Hospital, Paris, France.; Coiffard B; Department of Respiratory Medicine and Lung Transplantation, Assistance Publique - Hôpitaux de Marseille, Hôpital Nord; Aix-Marseille Université, Marseille, France.; Roux A; Department of Pulmonary Medicine, Foch Hospital, Suresnes, France.; Hirschi S; Respiratory Medicine and Lung Transplantation, Strasbourg University Hospital, Strasbourg, France.; Degot T; Respiratory Medicine and Lung Transplantation, Strasbourg University Hospital, Strasbourg, France.; Bunel V; AP-HP, Bichat Claude Bernard Hospital, Pulmonology B and Lung Transplant Department, INSERM 1152, Paris, France.; Le Pavec J; Service de Pneumologie et de Transplantation Pulmonaire, Hôpital Marie-Lannelongue, Groupe hospitalier Paris-Saint Joseph, Le Plessis-Robinson, France.; Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France.; UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France.; Macey J; Respiratory Medicine and Cystic Fibrosis Center, University Hospital Center of Bordeaux, Bordeaux, France.; Le Borgne A; Service de Pneumologie, Pôle des voies respiratoires, Hôpital Larrey, CHU Toulouse, Toulouse, France.; Legendre M; Assistance Publique - Hôpitaux de Paris (APHP) - Sorbonne Université, Inserm Childhood Genetic Disorders and Reference Center for Rare Lung Diseases, Armand Trousseau Hospital, Paris, France.; UF de Génétique moléculaire, APHP, Sorbonne Université, Hôpital Armand-Trousseau, Paris, France.; Cottin V; Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon; IVPC, INRAE, Claude Bernard University Lyon 1, member of ERN-LUNG, Lyons, France.; Thomas PA; Department of Thoracic Surgery, North Hospital, Aix-Marseille University, CNRS, INSERM, CRCM, AP-HM, Chemin des Bourrely, Marseille, France.; Borie R; Service de Pneumologie A, APHP, Hôpital Bichat, Université de Paris and INSERM U1152, Paris, France.; Reynaud-Gaubert M; Department of Respiratory Medicine and Lung Transplantation, Assistance Publique - Hôpitaux de Marseille, Hôpital Nord; Aix-Marseille Université, Marseille, France.
Source
Publisher: European Respiratory Society Country of Publication: England NLM ID: 101671641 Publication Model: eCollection Cited Medium: Print ISSN: 2312-0541 (Print) Linking ISSN: 23120541 NLM ISO Abbreviation: ERJ Open Res Subsets: PubMed not MEDLINE
Subject
Language
English
ISSN
2312-0541
Abstract
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 SFTPA1 (n=5), SFTPA2 (n=7) or SFTPC (n=8) were included. Median interquartile range (IQR) age at diagnosis was 45 (40-48) years, and median (IQR) age at lung transplantation was 51 (45-54) years. Median overall survival after transplantation was 8.6 years. Two patients had a pre-transplant history of lung cancer, and two developed post-transplant lung cancer. Female gender and a body mass index <25 kg·m-2 were significantly associated with a better prognosis, whereas transplantation in high emergency was associated with a worst prognosis.
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.
Competing Interests: 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.
(Copyright ©The authors 2023.)
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 SFTPA1 (n=5), SFTPA2 (n=7) or SFTPC (n=8) were included. Median interquartile range (IQR) age at diagnosis was 45 (40-48) years, and median (IQR) age at lung transplantation was 51 (45-54) years. Median overall survival after transplantation was 8.6 years. Two patients had a pre-transplant history of lung cancer, and two developed post-transplant lung cancer. Female gender and a body mass index <25 kg·m
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.
Competing Interests: 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.
(Copyright ©The authors 2023.)