Jérôme Le Pavec1,2,3, Dominique Valeyre4,5,6, Pierre Gazengel1,2,3,6, Are M Holm7,6, Hans Henrik Schultz8,6, Michael Perch8, Aurélie Le Borgne9, Martine Reynaud-Gaubert10, Christiane Knoop11, Laurent Godinas12, Sandrine Hirschi13, Vincent Bunel14, Rosalia Laporta15, Sergio Harari16, Elodie Blanchard17, Jesper M Magnusson18, Adrien Tissot19, Jean-François Mornex20,21, Clément Picard22, Laurent Savale2,3,23, Jean-François Bernaudin24, Pierre-Yves Brillet25, Hilario Nunes4, Marc Humbert2,3,23, Elie Fadel1,2,3, Jens Gottlieb26. 1. Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France. 2. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France. 3. UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France. 4. INSERM UMR 1272, Université Sorbonne Paris Nord, AP-HP, Hôpital Avicenne Service de Pneumologie, Bobigny, France. 5. Groupe Hospitalier Paris Saint Joseph, Paris, France. 6. These authors contributed equally to this work. 7. Dept of Respiratory Medicine, Oslo University Hospital and Institute for Clinical Medicine University of Oslo, Oslo, Norway. 8. Dept of Cardiology, Section for Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 9. Pôle des voies respiratoires-Hôpital Larrey, Centre Hopitalo-Universitaire, Toulouse, France. 10. Service de Pneumologie et Equipe de Transplantation Pulmonaire, Centre Hospitalo-Universitaire Nord, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France. 11. Brussels Lung Transplant Program, Dept of Chest Medicine, Erasme University, Brussels, Belgium. 12. Dept of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium. 13. Service de Pneumologie, Groupe de Transplantation Pulmonaire, Hopitaux Universitaires de Strasbourg, Strasbourg, France. 14. AP-HP, Service de Pneumologie, Hôpital Bichat, Paris, France. 15. Pneumology Dept, Hospital Universitario Puerta de Hierro, Madrid, Spain. 16. Dept of Medical Sciences San Giuseppe Hospital MultiMedica IRCCS and Dept of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy. 17. Dept of Respiratory Medicine, Haut-Lévèque Hospital, Bordeaux University, Pessac, France. 18. Dept of Internal Medicine/Respiratory Medicine and Allergology, Institute of Medicine, University of Gothenburg, Gothenburg, Sweden. 19. Service de Pneumologie, L'institut du thorax, Centre hospitalo-universitaire de Nantes, Nantes, France. 20. Université de Lyon, INRA, IVPC, Lyon, France. 21. Hospices Civils de Lyon, Lyon, France. 22. Service de Pneumologie, Hôpital Foch, Suresnes, France. 23. AP-HP, Service de Pneumologie, Hôpital Kremlin Bicêtre, Kremlin Bicêtre, France. 24. INSERM UMR 1272, Université Sorbonne Paris Nord, Bobigny and Sorbonne Université Paris, Paris, France. 25. INSERM U1272, Université Paris Sorbonne Nord, AP-HP, Service de Radiologie, Hôpital Avicenne, Bobigny, France. 26. Dept of Respiratory Medicine, Hannover Medical School, Hanover, Germany.
Abstract
STUDY QUESTION: In patients with sarcoidosis, past and ongoing immunosuppressive regimens, recurrent disease in the transplant and extrapulmonary involvement may affect outcomes of lung transplantation. We asked whether sarcoidosis lung phenotypes can be differentiated and, if so, how they relate to outcomes in patients with pulmonary sarcoidosis treated by lung transplantation. PATIENTS AND METHODS: We retrospectively reviewed data from 112 patients who met international diagnostic criteria for sarcoidosis and underwent lung or heart-lung transplantation between 2006 and 2019 at 16 European centres. RESULTS: Patient survival was the main outcome measure. At transplantation, median (interaquartile range (IQR)) age was 52 (46-59) years; 71 (64%) were male. Lung phenotypes were individualised as follows: 1) extended fibrosis only; 2) airflow obstruction; 3) severe pulmonary hypertension (sPH) and airflow obstruction; 4) sPH, airflow obstruction and fibrosis; 5) sPH and fibrosis; 6) airflow obstruction and fibrosis; 7) sPH; and 8) none of these criteria, in 17%, 16%, 17%, 14%, 11%, 9%, 5% and 11% of patients, respectively. Post-transplant survival rates after 1, 3, and 5 years were 86%, 76% and 69%, respectively. During follow-up (median (IQR) 46 (16-89) months), 31% of patients developed chronic lung allograft dysfunction. Age and extended lung fibrosis were associated with increased mortality. Pulmonary fibrosis predominating peripherally was associated with short-term complications. ANSWER TO THE STUDY QUESTION: Post-transplant survival in patients with pulmonary sarcoidosis was similar to that in patients with other indications for lung transplantation. The main factors associated with worse survival were older age and extensive pre-operative lung fibrosis.
STUDY QUESTION: In patients with sarcoidosis, past and ongoing immunosuppressive regimens, recurrent disease in the transplant and extrapulmonary involvement may affect outcomes of lung transplantation. We asked whether sarcoidosis lung phenotypes can be differentiated and, if so, how they relate to outcomes in patients with pulmonary sarcoidosis treated by lung transplantation. PATIENTS AND METHODS: We retrospectively reviewed data from 112 patients who met international diagnostic criteria for sarcoidosis and underwent lung or heart-lung transplantation between 2006 and 2019 at 16 European centres. RESULTS:Patient survival was the main outcome measure. At transplantation, median (interaquartile range (IQR)) age was 52 (46-59) years; 71 (64%) were male. Lung phenotypes were individualised as follows: 1) extended fibrosis only; 2) airflow obstruction; 3) severe pulmonary hypertension (sPH) and airflow obstruction; 4) sPH, airflow obstruction and fibrosis; 5) sPH and fibrosis; 6) airflow obstruction and fibrosis; 7) sPH; and 8) none of these criteria, in 17%, 16%, 17%, 14%, 11%, 9%, 5% and 11% of patients, respectively. Post-transplant survival rates after 1, 3, and 5 years were 86%, 76% and 69%, respectively. During follow-up (median (IQR) 46 (16-89) months), 31% of patients developed chronic lung allograft dysfunction. Age and extended lung fibrosis were associated with increased mortality. Pulmonary fibrosis predominating peripherally was associated with short-term complications. ANSWER TO THE STUDY QUESTION: Post-transplant survival in patients with pulmonary sarcoidosis was similar to that in patients with other indications for lung transplantation. The main factors associated with worse survival were older age and extensive pre-operative lung fibrosis.
Authors: Tim Oqueka; Sören Galow; Marcel Simon; Anna Weidmann; Nicole Stübiger; Elvin Zengin-Sahm; Christoph Sinning; Martin Krusche; Nikolas Ruffer; Stefan Steurer; Xenia Schick-Bengardt; Marcial Sebode; Ludwig Jesse Horst; Oliver M Steinmetz; Simon Melderis; Sina Cathérine Rosenkranz; Katharina Möller; Holger Jantke; Hans Klose Journal: Z Rheumatol Date: 2022-08-04 Impact factor: 1.530