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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. 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. 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. 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. Copyright ©The authors 2021. For reproduction rights and permissions contact permissions@ersnet.org.

Citation

Jérôme Le Pavec, Dominique Valeyre, Pierre Gazengel, Are M Holm, Hans Henrik Schultz, Michael Perch, Aurélie Le Borgne, Martine Reynaud-Gaubert, Christiane Knoop, Laurent Godinas, Sandrine Hirschi, Vincent Bunel, Rosalia Laporta, Sergio Harari, Elodie Blanchard, Jesper M Magnusson, Adrien Tissot, Jean-François Mornex, Clément Picard, Laurent Savale, Jean-François Bernaudin, Pierre-Yves Brillet, Hilario Nunes, Marc Humbert, Elie Fadel, Jens Gottlieb. Lung transplantation for sarcoidosis: outcome and prognostic factors. The European respiratory journal. 2021 Aug;58(2)

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PMID: 33479107

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