Pauline Pradère1, Igor Tudorache2, Jesper Magnusson3, Laurent Savale4, Olivier Brugiere5, Benoît Douvry6, Martine Reynaud-Gaubert7, Johanna Claustre8, Aurélie Le Borgne9, Are M Holm10, Hans Henrik Schultz11, Christiane Knoop12, Laurent Godinas13, Andrew J Fisher14, Sandrine Hirschi15, Jens Gottlieb16, Jérôme Le Pavec17. 1. Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, 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. 2. Department of Cardiothoracic, Transplant and Vascular Surgery, Hannover Medical School, Hannover, Germany. 3. Department of Internal Medicine/Respiratory Medicine and Allergology, Institute of Medicine, University of Gothenburg, Gothenburg, Sweden. 4. 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; AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. 5. AP-HP, Service de Pneumologie, Hôpital Bichat, Paris, France. 6. Service de Pneumologie, Hôpital Foch, Suresnes, France. 7. Service de Pneumologie et Equipe de Transplantation Pulmonaire, Centre Hospitalo-Universitaire Nord, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France. 8. Clinique Universitaire de Pneumologie, Centre Hopitalo-Universitaire, Grenoble, France. 9. Pôles des voies respiratoires-Hôpital Larrey, Centre Hopitalo-Universitaire, Toulouse, France. 10. Department of Respiratory Medicine Oslo University Hospital and Institute for Clinical Medicine University of Oslo, Oslo, Norway. 11. Department of Cardiology, Section for Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 12. Brussels Lung Transplant Program, Department of Chest Medicine, Erasme University, Brussels, Belgium. 13. Service de Pneumologie, Centre Hopitalo-Universitaire UCL Namur, Namur, Belgium. 14. Institute of Transplantation, Newcastle upon Tyne Hospitals NHS Trust and Institute of Cellular Medicine, Newcastle University, Newcastle, UK. 15. Service de Pneumologie, Groupe de Transplantation Pulmonaire, Hopitaux Universitaires de Strasbourg, Strasbourg, France. 16. Department of Respiratory Medicine, Hannover Medical School and Biomedical Research in End-stage and Obstructive Disease, German Centre for Lung Research, Hannover, Germany. 17. Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, 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. Electronic address: lepavec@gmail.com.
Abstract
BACKGROUND: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Our objective in this study was to assess the indications, survival, and prognostic factors of lung or heart-lung transplantation for scleroderma lung disease. METHODS: We retrospectively reviewed the data of 90 patients with scleroderma who underwent lung or heart-lung transplantation between 1993 and 2016 at 14 European centers. International criteria were used to diagnose scleroderma. Pulmonary hypertension (PH) was diagnosed during right heart catheterization based on international guidelines. RESULTS: Survival rates after 1, 3, and 5 years were 81%, 68%, and 61%, respectively. By univariate analysis, borderline-significant associations with poorer survival were found for female gender (hazard ratio 2.11; 95% confidence interval [CI] 0.99 to 4.50; p = 0.05) and PAH as the reason for transplantation (hazard ratio 1.90; 95% CI 0.96 to 3.92; p = 0.06). When both these factors were present in combination, the risk of death was 3-fold that in males without PAH. The clinical and histologic presentation resembled veno-occlusive disease in 75% of patients with PAH. CONCLUSIONS: Post-transplant survival rates and freedom from chronic lung allograft dysfunction in patients with scleroderma were similar to those in patients with other reasons for lung transplantation. Female sex and PAH in combination was associated with lower survival.
BACKGROUND: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Our objective in this study was to assess the indications, survival, and prognostic factors of lung or heart-lung transplantation for scleroderma lung disease. METHODS: We retrospectively reviewed the data of 90 patients with scleroderma who underwent lung or heart-lung transplantation between 1993 and 2016 at 14 European centers. International criteria were used to diagnose scleroderma. Pulmonary hypertension (PH) was diagnosed during right heart catheterization based on international guidelines. RESULTS: Survival rates after 1, 3, and 5 years were 81%, 68%, and 61%, respectively. By univariate analysis, borderline-significant associations with poorer survival were found for female gender (hazard ratio 2.11; 95% confidence interval [CI] 0.99 to 4.50; p = 0.05) and PAH as the reason for transplantation (hazard ratio 1.90; 95% CI 0.96 to 3.92; p = 0.06). When both these factors were present in combination, the risk of death was 3-fold that in males without PAH. The clinical and histologic presentation resembled veno-occlusive disease in 75% of patients with PAH. CONCLUSIONS: Post-transplant survival rates and freedom from chronic lung allograft dysfunction in patients with scleroderma were similar to those in patients with other reasons for lung transplantation. Female sex and PAH in combination was associated with lower survival.
Authors: Miguel M Leiva-Juárez; Andreacarola Urso; Joseph Costa; Bryan P Stanifer; Joshua R Sonett; Luke Benvenuto; Megan Aversa; Hilary Robbins; Lori Shah; Selim Arcasoy; Frank D'Ovidio Journal: J Scleroderma Relat Disord Date: 2021-05-25
Authors: Alicia M Hinze; Cheng T Lin; Amira F Hussien; Jamie Perin; Aida Venado; Jeffrey A Golden; Francesco Boin; Robert H Brown; Robert A Wise; Fredrick M Wigley Journal: Rheumatology (Oxford) Date: 2020-04-01 Impact factor: 7.580
Authors: Jake G Natalini; Joshua M Diamond; Mary K Porteous; David J Lederer; Keith M Wille; Ann B Weinacker; Jonathan B Orens; Pali D Shah; Vibha N Lama; John F McDyer; Laurie D Snyder; Chadi A Hage; Jonathan P Singer; Lorraine B Ware; Edward Cantu; Michelle Oyster; Laurel Kalman; Jason D Christie; Steven M Kawut; Elana J Bernstein Journal: J Heart Lung Transplant Date: 2021-01-23 Impact factor: 10.247