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2. Blood MMP-9 measured at 2 years after lung transplantation as a prognostic biomarker of chronic lung allograft dysfunction

Author

Tissot, Adrien, Durand, Eugénie, Goronflot, Thomas, Coiffard, Benjamin, Renaud-Picard, Benjamin, Roux, Antoine, Demant, Xavier, Mornex, Jean-François, Falque, Loïc, Salpin, Mathilde, Le Pavec, Jérôme, Villeneuve, Thomas, Boussaud, Véronique, Knoop, Christiane, Magnan, Antoine, Lair, David, Berthelot, Laureline, Danger, Richard, and Brouard, Sophie

Published
2024
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3. Description and first insights on a large genomic biobank of lung transplantation

Author

Brocard, Simon, Morin, Martin, dos Santos Brito Silva, Nayane, Renaud-Picard, Benjamin, Coiffard, Benjamin, Demant, Xavier, Falque, Loïc, Le Pavec, Jérome, Roux, Antoine, Villeneuve, Thomas, Knoop, Christiane, Mornex, Jean-François, Salpin, Mathilde, Boussaud, Véronique, Rousseau, Olivia, Mauduit, Vincent, Durand, Axelle, Magnan, Antoine, Gourraud, Pierre-Antoine, Vince, Nicolas, Südholt, Mario, Tissot, Adrien, and Limou, Sophie

Published
2024
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7. 2022 Update of indications and contraindications for lung transplantation in France

Author

Le Pavec, Jérôme, Pison, Christophe, Hirschi, Sandrine, Bunel, Vincent, Mordant, Pierre, Brugière, Olivier, Guen, Morgan Le, Olland, Anne, Coiffard, Benjamin, Renaud-Picard, Benjamin, Tissot, Adrien, Brioude, Geoffrey, Borie, Raphaël, Crestani, Bruno, Deslée, Gaétan, Stelianides, Sandrine, Mal, Hervé, Schuller, Armelle, Falque, Loïc, Lorillon, Gwenaëlle, Tazi, Abdellatif, Burgel, Pierre Regis, Grenet, Dominique, De Miranda, Sandra, Bergeron, Anne, Launay, David, Cottin, Vincent, Nunes, Hilario, Valeyre, Dominique, Uzunhan, Yurdagul, Prévot, Grégoire, Sitbon, Olivier, Montani, David, Savale, Laurent, Humbert, Marc, Fadel, Elie, Mercier, Olaf, Mornex, Jean François, Dauriat, Gaëlle, and Reynaud-Gaubert, Martine

Published
2023
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9. Lung transplantation for interstitial lung disease in idiopathic inflammatory myositis: A cohort study

Author

Rivière, Amélie, Picard, Clément, Berastegui, Cristina, Mora, Victor Manuel, Bunel, Vincent, Godinas, Laurent, Salvaterra, Elena, Rossetti, Valeria, Savale, Laurent, Israel-Biet, Dominique, Demant, Xavier, Bermudez, Julien, Meloni, Federica, Jaksch, Peter, Magnusson, Jesper, Beaumont, Laurence, Perch, Michael, Mornex, Jean-François, Knoop, Christiane, Aubert, John-David, Hervier, Baptiste, Nunes, Hilario, Humbert, Marc, Gottlieb, Jens, Uzunhan, Yurdagul, and Le Pavec, Jérôme

Published
2022
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10. Chronic lung allograft dysfunction is associated with an early increase of circulating cytotoxic CD4+CD57+ILT2+ T cells, selectively inhibited by the immune check-point HLA-G

Author

Brugière, Olivier, Mouren, Domitille, Trichereau, Julie, Vallée, Alexandre, Kuzniak, Isabelle, Hirschi, Sandrine, Renaud-Picard, Benjamin, Reynaud-Gaubert, Martine, Nieves, Ana, Bunel, Vincent, Messika, Jonathan, Demant, Xavier, Macey, Julie, Le Pavec, Jérôme, Dauriat, Gaëlle, Saint-Raymond, Christel, Falque, Loic, Mornex, Jean-François, Tissot, Adrien, Foureau, Aurore, Borgne Krams, Aurélie Le, Bousseau, Véronique, Magnan, Antoine, Picard, Clément, Roux, Antoine, Carosella, Edgardo, LeMaoult, Joel, and Rouas-Freiss, Nathalie

Published
2022
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11. Prospective Multicenter Validation of the Detection of ALK Rearrangements of Circulating Tumor Cells for Noninvasive Longitudinal Management of Patients With Advanced NSCLC

Author

Israel-Biet, Dominique, Pison, Christophe, Moro-Sibilot, Denis, Lantuejoul, Sylvie, Stephanov, Olivier, Juliette, Meyzenc, Mendozat, Christophe, Zaidi, Manel, Coulouvrat, Sandra, Col, Edwige, Chanez, Pascal, Greillier, Laurent, Tomasini, Pascale, Barlesi, Fabrice, Mascaux, Céline, Jourdan, Sandrine, Roger, Aurélie, Biemar, Julie, Randriamampionona, Rondro, Chabot, François, Tiotiu, Angélica, Clement-Duchene, Christelle, Vignaud, Jean-Michel, Lacomme, Stéphanie, Lomazzi, Sandra, Laurent, Carine, Bulsei, Xavier, Bischoff, Laura, Rakotonirina, Raymond, Layouni, Mehdi, Deslee, Gaëtan, Mal, Hervé, Kessler, Romain, Vergnon, Jean-Michel, Pelissier, Isabelle, Cuvelier, Antoine, Bourdin, Arnaud, Jounieaux, Vincent, Roche, Nicolas, Jouneau, Stéphane, Bonniaud, Philippe, Scherpereel, Arnaud, Mornex, Jean François, Steenhouwer, François, Leroy, Sylvie, Marquette, Charles Hugo, Benzaquen, Jonathan, Mazzette, Andrea, Padovani, Bernard, Hofman, Paul, Ilié, Marius, Hofman, Véronique, Fayada, Julien, Long-Mira, Elodie, Lassalle, Sandra, Pradelli, Johanna, Martinez, Estelle, Habault, Marine, Bonnard, Mélanie, Moutarde, Julie, Yatimi, Rachida, Labsi, Hakima, Gazoppi, Loïc, Lpce, Tumorothèque, Griffonnet, Jennifer, Fontaine, Maureen, Guillemart, Ariane, Butori, Catherine, Selva, Eric, Poudenx, Michel, Otto, Josiane, Hebert, Christophe, Botchiellini, Delphine, Boudouf, Soukaina, Menier, Margaux, Occeli, Estelle, Bellentani, Sophie, Pion, Carine, Fournier, Elodie, Thariat, Juliette, Gervais, Radj, Hamond, Karim, Marchand-Adam, Sylvain, Plantier, Laurent, Fajolle, Gaelle, Rayez, Mélanie, Cadranel, Jacques, Fallet, Vincent, Wislez, Marie, Antoine, Martine, Cote, Jean-François, Chaabane, Nouha, Ruppert, Anne Marie, Bertrand, Eliane, Rodenas, Anita, Pontdeme, Gwenaëlle, Mathiot, Nathalie, Ribert, Tamazouzt, Mazières, Julien, Guibert, Nicolas, Rouviere, Damien, Bousquet, Emilie, Bigay-Game, Laurence, Hermant, Christophe, Plat, Gavin, Rouquette, Isabelle, Evrard, Solène, Gouin, Sandrine, Clermont, Estelle Taranchon, Dormoy, Inge, Coulomb, Christelle, Pradine, Anne, Lambert, Véronique, Laborde, Lilian, Castelnau, Olivier, Chamorey, Emmanuel, Heeke, Simon, Thamphya, Brice, Boutros, Jacques, Clément-Duchêne, Christelle, and Marquette, Charles-Hugo

Published
2021
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14. Circulating tumour cells as a potential biomarker for lung cancer screening: a prospective cohort study

Author

MARQUETTE, Charles-Hugo, BOUTROS, Jacques, Benzaquen, Jonathan, FERREIRA, Marion, PASTRE, Jean, Pison, Christophe, PADOVANI, Bernard, BETTAYEB, Faiza, FALLET, Vincent, GUIBERT, Nicolas, BASILLE, Damien, ILIE, Marius, HOFMAN, Véronique, HOFMAN, Paul, ISRAEL-BIET, Dominique, CHABOT, François, GUILLAUMOT, Anne, DESLEE, Gaetan, PEROTIN, Jeanne-Marie, DURY, Sandra, MAL, Hervé, MARCEAU, Armelle, Kessler, Romain, Vergnon, Jean-Michel, Pelissier, Carole, Di Palma, Fabrice, Cuvelier, Antoine, PATOUT, Maxime, Bourdin, Arnaud, GAMEZ, Anne Sophie, ANDREJAK, Claire, POULET, Claire, FRANCOIS, Géraldine, Jounieaux, Vincent, Roche, Nicolas, Jouneau, Stéphane, Brinchault, Graziella, Bonniaud, Philippe, ZOUAK, Ayoub, Scherpereel, Arnaud, BALDACCI, Simon, CORTOT, Alexis, Mornex, Jean François, Steenhouwer, François, LEROY, Sylvie, BERTHET, Jean-Philippe, FONTAS, Eric, BULSEI, Julie, CRUZEL, Coralie, Pradelli, Johanna, Fontaine, Maureen, MANIEL, Charlotte, Griffonnet, Jennifer, BUTORI, Catherine, SELVA, Eric, POUDENX, Michel, AguilanIu, Bernard, Ferretti, Gilbert, Arbib, François, Briault, Amandine, Toffart, Anne-Claire, Dahalani, Raissa, Destors, Marie, Chanez, Pascal, GREILLIER, Laurent, ASTOUL, Philippe, BARLESI, Fabrice, GAUBERT, Jean-Yves, Mazières, Julien, Marchand-Adam, Sylvain, Cadranel, Jacques, CHAABANE, Nouha, IZADIFAR, Armine, ROSENCHER, Lise, RUPPERT, Anne-Marie, VIEIRA, Thibault, MATHIOT, Nathalie, Marquette, Charles-Hugo, Boutros, Jacques, Ferreira, Marion, Pastre, Jean, Padovani, Bernard, Bettayeb, Faiza, Fallet, Vincent, Guibert, Nicolas, Basille, Damien, Ilie, Marius, Hofman, Véronique, and Hofman, Paul

Published
2020
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16. High circulating CD4+CD25hiFOXP3+ T-cell sub-population early after lung transplantation is associated with development of bronchiolitis obliterans syndrome

Author

Durand, Maxim, Lacoste, Philippe, Danger, Richard, Jacquemont, Lola, Brosseau, Carole, Durand, Eugénie, Tilly, Gaelle, Loy, Jennifer, Foureau, Aurore, Royer, Pierre-Joseph, Tissot, Adrien, Roux, Antoine, Reynaud-Gaubert, Martine, Kessler, Romain, Mussot, Sacha, Dromer, Claire, Brugière, Olivier, Mornex, Jean François, Guillemain, Romain, Claustre, Johanna, Degauque, Nicolas, Magnan, Antoine, and Brouard, Sophie

Published
2018
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17. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus: Results From the French Pulmonary Hypertension Registry

Author

Balquet, Marie-Hélène, Ziza, Jean-Marc, Clauvel, Jean-Pierre, Brouet, Jean-Claude, Pison, Christophe, Chabot, Jean-François, Velly, Jean-François, Dos Santos, Pierre-Dominique, Meurice, Jean-Claude, Fauchais, Anne-Laure, Guillevin, Loïc, Cadranel, Jacques, Traclet, Julie, Mornex, Jean-François, Mabo, Philippe, Didier, Alain, Hachulla, Eric, Jais, Xavier, Cinquetti, Gaël, Clerson, Pierre, Rottat, Laurence, Launay, David, Cottin, Vincent, Habib, Gilbert, Prevot, Grégoire, Chabanne, Céline, Foïs, Eléna, Amoura, Zahir, Mouthon, Luc, Le Guern, Véronique, Montani, David, Simonneau, Gérald, Humbert, Marc, Sobanski, Vincent, and Sitbon, Olivier

Published
2018
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18. Effect of antifibrotic agents on postoperative complications after lung transplantation for idiopathic pulmonary fibrosis

Author

Moncomble, Elsa, primary, Weisenburger, Gaelle, additional, Picard, Clément, additional, Dégot, Tristan, additional, Reynaud‐Gaubert, Martine, additional, Nieves, Ana, additional, Mornex, Jean François, additional, Dauriat, Gaelle, additional, Messika, Jonathan, additional, Godet, Cendrine, additional, Hirschi, Sandrine, additional, Le Pavec, Jérôme, additional, Borie, Raphael, additional, Mordant, Pierre, additional, Lortat‐Jacob, Brice, additional, Mal, Hervé, additional, and Bunel, Vincent, additional

Published
2023
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19. Bioreactance assessment of cardiac output lacks reliability for the follow-up of patients with pulmonary hypertension.

Author

Turquier, Ségolène, Huot, Laure, Lamkhioued, Medhi, Subtil, Fabien, Traclet, Julie, Ahmad, Kais, Lestelle, François, Chauvelot, Louis, Cottin, Vincent, and Mornex, Jean-François

Subjects
CARDIAC output, PULMONARY hypertension, HYPERTENSION, BLAND-Altman plot, RANK correlation (Statistics), CARDIAC catheterization, THERMOCYCLING
Abstract

Cardiac output (CO) is one of the primary prognostic factors evaluated during the follow-up of patients treated for pulmonary hypertension (PH). It is recommended that it be measured using the thermodilution technique during right heart catheterization. The difficulty to perform iterative invasive measurements on the same individual led us to consider a non-invasive option. The aims of the present study were to assess the agreement between CO values obtained using bioreactance (Starling™ SV) and thermodilution, and to evaluate the ability of the bioreactance monitor to detect patients whose CO decreased by more than 15% during follow-up and, accordingly, its usefulness for patient monitoring. A prospective cohort study evaluating the performance of the Starling™ SV monitor was conducted in patients with clinically stable PH. Sixty patients referred for hemodynamic assessment were included. CO was measured using both the thermodilution technique and bioreactance during two follow-up visits. A total of 60 PH patients were included. All datasets were available at the baseline visit (V0) and 50 of them were usable during the follow-up visit (V1). Median [IQR] CO was 4.20 l/min [3.60–4.70] when assessed by bioreactance, and 5.30 l/min [4.57–6.20] by thermodilution (p<0.001). The Spearman correlation coefficient was 0.51 [0.36–0.64], and the average deviation on Bland-Altman plot was -1.25 l/min (95% CI [-1.48–1.01], p<0.001). The ability of the monitor to detect a variation in CO of more than 15% between two follow-up measurements, when such variation existed using thermodilution, was insufficient for clinical practice (AUC = 0.54, 95% CI [0.33–0.75]). [ABSTRACT FROM AUTHOR]

Published
2024
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21. Effect of antifibrotic agents on postoperative complications after lung transplantation for idiopathic pulmonary fibrosis.

Author

Moncomble, Elsa, Weisenburger, Gaelle, Picard, Clément, Dégot, Tristan, Reynaud‐Gaubert, Martine, Nieves, Ana, Mornex, Jean François, Dauriat, Gaelle, Messika, Jonathan, Godet, Cendrine, Hirschi, Sandrine, Le Pavec, Jérôme, Borie, Raphael, Mordant, Pierre, Lortat‐Jacob, Brice, Mal, Hervé, and Bunel, Vincent

Subjects
IDIOPATHIC pulmonary fibrosis, LUNG transplantation, SURGICAL complications, HEALING, OVERALL survival
Abstract

Background: Antifibrotic agents (AFAs) are now standard‐of‐care for idiopathic pulmonary fibrosis (IPF). Concerns have arisen about the safety of these drugs in patients undergoing lung transplantation (LTx). Methods: We performed a multi‐centre, nationwide, retrospective, observational study of French IPF patients undergoing LTx between 2011 and 2018 to determine whether maintaining AFAs in the peri‐operative period leads to increased bronchial anastomoses issues, delay in skin healing and haemorrhagic complications. We compared the incidence of post‐operative complications and the survival of patients according to AFA exposure. Results: Among 205 patients who underwent LTx for IPF during the study period, 58 (28%) had received AFAs within 4 weeks before LTx (AFA group): pirfenidone in 37 (18.0%) and nintedanib in 21 (10.2%). The median duration of AFA treatment before LTx was 13.8 (5.6–24) months. The AFA and control groups did not significantly differ in airway, bleeding or skin healing complications (p = 0.91, p = 0.12 and p = 0.70, respectively). Primary graft dysfunction was less frequent in the AFA than control group (26% vs. 43%, p = 0.02), and the 90‐day mortality was lower (7% vs. 18%, p = 0.046). Conclusions: AFA therapy did not increase airway, bleeding or wound post‐operative complications after LTx and could be associated with reduced rates of primary graft dysfunction and 90‐day mortality. [ABSTRACT FROM AUTHOR]

Published
2024
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22. Changes in HCMV immune cell frequency and phenotype are associated with chronic lung allograft dysfunction

Author

Rousselière, Amélie, primary, Delbos, Laurence, additional, Foureau, Aurore, additional, Reynaud-Gaubert, Martine, additional, Roux, Antoine, additional, Demant, Xavier, additional, Le Pavec, Jérôme, additional, Kessler, Romain, additional, Mornex, Jean-François, additional, Messika, Jonathan, additional, Falque, Loïc, additional, Le Borgne, Aurélie, additional, Boussaud, Véronique, additional, Tissot, Adrien, additional, Hombourger, Sophie, additional, Bressollette-Bodin, Céline, additional, and Charreau, Béatrice, additional

Published
2023
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23. Diffuse panniculitis in a teen male with ZZ alpha1-antitrypsin deficiency

Author

Papiris, Spyros A., primary, Parmaxidis, Anthimos, additional, Theotokoglou, Sofia, additional, Tsakiraki, Zoe, additional, Veith, Martina, additional, Panagiotou, Aikaterini, additional, Pappa, Vasiliki, additional, Kallieri, Maria, additional, Mornex, Jean-François, additional, Katoulis, Alexander C., additional, Haritos, Dionysios, additional, Panayiotides, Ioannis G., additional, and Manali, Effrosyni D., additional

Published
2023
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26. Donor Club Cell Secretory Protein G38A Polymorphism Is Associated With a Decreased Risk of Primary Graft Dysfunction in the French Cohort in Lung Transplantation

Author

Hin, Angela, Kannengiesser, Caroline, Roussel, Arnaud, Renaud-Picard, Benjamin, Roux, Antoine, Reynaud-Gaubert, Martine, Claustre, Johanna, Tissot, Adrien, Guillemain, Romain, Mornex, Jean-François, Mussot, Sacha, Dromer, Claire, Dahan, Marcel, Brugière, Olivier, Mercier, Olaf, Borie, Raphaël, Pretolani, Marina, Castier, Yves, and Mordant, Pierre

Published
2018
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27. Inverted direct allorecognition triggers early donor-specific antibody responses after transplantation

Author

Charmetant, Xavier, primary, Chen, Chien-Chia, additional, Hamada, Sarah, additional, Goncalves, David, additional, Saison, Carole, additional, Rabeyrin, Maud, additional, Rabant, Marion, additional, Duong van Huyen, Jean-Paul, additional, Koenig, Alice, additional, Mathias, Virginie, additional, Barba, Thomas, additional, Lacaille, Florence, additional, le Pavec, Jérôme, additional, Brugière, Olivier, additional, Taupin, Jean-Luc, additional, Chalabreysse, Lara, additional, Mornex, Jean-François, additional, Couzi, Lionel, additional, Graff-Dubois, Stéphanie, additional, Jeger-Madiot, Raphaël, additional, Tran-Dinh, Alexy, additional, Mordant, Pierre, additional, Paidassi, Helena, additional, Defrance, Thierry, additional, Morelon, Emmanuel, additional, Badet, Lionel, additional, Nicoletti, Antonino, additional, Dubois, Valérie, additional, and Thaunat, Olivier, additional

Published
2022
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30. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus: Results From the French Pulmonary Hypertension Registry

Author

Hachulla, Eric, Jais, Xavier, Cinquetti, Gaël, Clerson, Pierre, Rottat, Laurence, Launay, David, Cottin, Vincent, Habib, Gilbert, Prevot, Grégoire, Chabanne, Céline, Foïs, Eléna, Amoura, Zahir, Mouthon, Luc, Le Guern, Véronique, Montani, David, Simonneau, Gérald, Humbert, Marc, Sobanski, Vincent, Sitbon, Olivier, Balquet, Marie-Hélène, Ziza, Jean-Marc, Clauvel, Jean-Pierre, Brouet, Jean-Claude, Pison, Christophe, Chabot, Jean-François, Velly, Jean-François, Dos Santos, Pierre-Dominique, Meurice, Jean-Claude, Fauchais, Anne-Laure, Guillevin, Loïc, Cadranel, Jacques, Traclet, Julie, Mornex, Jean-François, Mabo, Philippe, and Didier, Alain

Published
2018
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32. Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort

Author

Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, and Borie, Raphaël

Published
2022
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34. Airway complications in lung transplant recipients with telomere-related interstitial lung disease.

Author

UCL - (SLuc) Service de pneumologie, Choi, Bina, Messika, Jonathan, Courtwright, Andrew, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Le Pavec, Jérôme, Quêtant, Sébastien, Froidure, Antoine, Lazor, Romain, Reynaud-Gaubert, Martine, Borgne, Aurélie Le, Houlbracq, Mathilde Phillips, Goldberg, Hilary, El-Chemaly, Souheil, Borie, Raphael, French Group of Lung Transplantation, UCL - (SLuc) Service de pneumologie, Choi, Bina, Messika, Jonathan, Courtwright, Andrew, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Le Pavec, Jérôme, Quêtant, Sébastien, Froidure, Antoine, Lazor, Romain, Reynaud-Gaubert, Martine, Borgne, Aurélie Le, Houlbracq, Mathilde Phillips, Goldberg, Hilary, El-Chemaly, Souheil, Borie, Raphael, and French Group of Lung Transplantation

Abstract

Patients with short telomere-related interstitial lung disease (ILD) have worse outcomes after lung transplantation. We hypothesized that post-transplant airway complications, including dehiscence and bronchial stenosis, would be more common in the short telomere ILD lung transplant population. We conducted a multi-institutional (Brigham and Women's Hospital, Groupe de Transplantation de la SPLF) retrospective cohort study of 63 recipients between 2009 and 2019 with ILD and short telomeres, compared to 4359 recipients from the Scientific Registry of Transplant Recipients with ILD and no known telomeropathy. In the short telomere cohort, six recipients (9.5%) developed dehiscence and nine recipients (14.3%) developed stenosis, compared to 60 (1.4%) and 149 (3.4%) in the control, respectively. After adjusting for age, sex, and bilaterality, the presence of short telomeres was associated with higher odds of dehiscence (odds ratio (OR) = 8.24, 95% confidence interval (CI) = 3.34 20.29, p < .001) and stenosis (OR = 4.63, 95% CI 2.21 9.69, p < .001). The association between the presence of short telomeres and post-transplant dehiscence and stenosis suggest that airway complications may be a contributor to increased morbidity and mortality in patients with telomere-related ILD.

Published
2022

35. Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.

Author

UCL - (SLuc) Service de pneumologie, Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, OrphaLung Network, UCL - (SLuc) Service de pneumologie, Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, and OrphaLung Network

Abstract

Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.

Published
2022

36. Lung transplantation for interstitial lung disease in idiopathic inflammatory myositis:A cohort study

Author

Rivière, Amélie, Picard, Clément, Berastegui, Cristina, Mora, Victor Manuel, Bunel, Vincent, Godinas, Laurent, Salvaterra, Elena, Rossetti, Valeria, Savale, Laurent, Israel-Biet, Dominique, Demant, Xavier, Bermudez, Julien, Meloni, Federica, Jaksch, Peter, Magnusson, Jesper, Beaumont, Laurence, Perch, Michael, Mornex, Jean François, Knoop, Christiane, Aubert, John David, Hervier, Baptiste, Nunes, Hilario, Humbert, Marc, Gottlieb, Jens, Uzunhan, Yurdagul, Le Pavec, Jérôme, Rivière, Amélie, Picard, Clément, Berastegui, Cristina, Mora, Victor Manuel, Bunel, Vincent, Godinas, Laurent, Salvaterra, Elena, Rossetti, Valeria, Savale, Laurent, Israel-Biet, Dominique, Demant, Xavier, Bermudez, Julien, Meloni, Federica, Jaksch, Peter, Magnusson, Jesper, Beaumont, Laurence, Perch, Michael, Mornex, Jean François, Knoop, Christiane, Aubert, John David, Hervier, Baptiste, Nunes, Hilario, Humbert, Marc, Gottlieb, Jens, Uzunhan, Yurdagul, and Le Pavec, Jérôme

Abstract

In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data for 64 patients who underwent lung transplantation between 2009 and 2021 at 19 European centers. Patient survival was the primary outcome. At transplantation, the median age was 53 [46–59] years, 35 (55%) patients were male, 31 (48%) had classical IIM, 25 (39%) had rapidly progressive ILD, and 21 (33%) were in a high-priority transplant allocation program. Survival rates after 1, 3, and 5 years were 78%, 73%, and 70%, respectively. During follow-up (median, 33 [7–63] months), 23% of patients developed chronic lung allograft dysfunction. Compared to amyopathic IIM, classical IIM was characterized by longer disease duration, higher-intensity immunosuppression before transplantation, and significantly worse posttransplantation survival. Five (8%) patients had a clinical IIM relapse, with mild manifestations. No patient experienced ILD recurrence in the allograft. Posttransplantation survival in IIM-ILD was similar to that in international all-cause-transplantation registries. The main factor associated with worse survival was a history of muscle involvement (classical IIM). In lung transplant recipients with idiopathic inflammatory myopathy, survival was similar to that in all-cause transplantation and was worse in patients with muscle involvement compared to those with the amyopathic disease.

Published
2022

41. Efficacy and Safety of Rovalpituzumab Tesirine Compared With Topotecan as Second-Line Therapy in DLL3-High SCLC: Results From the Phase 3 TAHOE Study

Author

Ilié, Marius, Mazières, Julien, Chamorey, Emmanuel, Heeke, Simon, Benzaquen, Jonathan, Thamphya, Brice, Boutros, Jacques, Tiotiu, Angélica, Fayada, Julien, Cadranel, Jacques, Poudenx, Michel, Moro-Sibilot, Denis, Barlesi, Fabrice, Thariat, Juliette, Clément-Duchêne, Christelle, Tomasini, Pascale, Hofman, Véronique, Marquette, Charles-Hugo, Hofman, Paul, Israel-Biet, Dominique, Pison, Christophe, Lantuejoul, Sylvie, Stephanov, Olivier, Juliette, Meyzenc, Mendozat, Christophe, Zaidi, Manel, Coulouvrat, Sandra, Col, Edwige, Chanez, Pascal, Greillier, Laurent, Mascaux, Céline, Jourdan, Sandrine, Roger, Aurélie, Biemar, Julie, Randriamampionona, Rondro, Chabot, François, Vignaud, Jean-Michel, Lacomme, Stéphanie, Lomazzi, Sandra, Laurent, Carine, Bulsei, Xavier, Bischoff, Laura, Rakotonirina, Raymond, Layouni, Mehdi, Deslee, Gaëtan, Mal, Hervé, Kessler, Romain, Vergnon, Jean-Michel, Pelissier, Isabelle, Cuvelier, Antoine, Bourdin, Arnaud, Jounieaux, Vincent, Roche, Nicolas, Jouneau, Stéphane, Bonniaud, Philippe, Scherpereel, Arnaud, Mornex, Jean François, Steenhouwer, François, Leroy, Sylvie, Marquette, Charles Hugo, Mazzette, Andrea, Padovani, Bernard, Long-Mira, Elodie, Lassalle, Sandra, Pradelli, Johanna, Martinez, Estelle, Habault, Marine, Bonnard, Mélanie, Moutarde, Julie, Yatimi, Rachida, Labsi, Hakima, Gazoppi, Loïc, Lpce, Tumorothèque, Griffonnet, Jennifer, Fontaine, Maureen, Guillemart, Ariane, Butori, Catherine, Selva, Eric, Otto, Josiane, Hebert, Christophe, Botchiellini, Delphine, Boudouf, Soukaina, Menier, Margaux, Occeli, Estelle, Bellentani, Sophie, Pion, Carine, Fournier, Elodie, Gervais, Radj, Hamond, Karim, Marchand-Adam, Sylvain, Plantier, Laurent, Fajolle, Gaelle, Rayez, Mélanie, Fallet, Vincent, Wislez, Marie, Antoine, Martine, Cote, Jean-François, Chaabane, Nouha, Ruppert, Anne Marie, Bertrand, Eliane, Rodenas, Anita, Pontdeme, Gwenaëlle, Mathiot, Nathalie, Ribert, Tamazouzt, Guibert, Nicolas, Rouviere, Damien, Bousquet, Emilie, Bigay-Game, Laurence, Hermant, Christophe, Plat, Gavin, Rouquette, Isabelle, Evrard, Solène, Gouin, Sandrine, Clermont, Estelle Taranchon, Dormoy, Inge, Coulomb, Christelle, Pradine, Anne, Lambert, Véronique, Laborde, Lilian, Castelnau, Olivier, University of Manchester [Manchester], Hôpital du Sacré-Coeur de Montréal, Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance Publique - Hôpitaux de Marseille (APHM), Yonsei University College of Medicine [Séoul, Corée du Sud], August Pi i Sunyer Biomedical Research Institute - IDIBAPS [Barcelona, Spain], Hospital de la Santa Creu i Sant Pau, University Hospitals Leuven [Leuven], National and Kapodistrian University of Athens (NKUA), Odense University Hospital (OUH), Oregon Health and Science University [Portland] (OHSU), Kyushu University, Hacettepe University = Hacettepe Üniversitesi, University of Copenhagen = Københavns Universitet (UCPH), University of Groningen [Groningen], Abbvie Inc. [North Chicago], Asklepios Klinikum Uckermark GmbH, Méthodes computationnelles pour la prise en charge thérapeutique en oncologie : Optimisation des stratégies par modélisation mécaniste et statistique (COMPO), Inria Sophia Antipolis - Méditerranée (CRISAM), Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre de Recherche en Cancérologie de Marseille (CRCM), and Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Institut Paoli-Calmettes

Subjects
0301 basic medicine, Pulmonary and Respiratory Medicine, Oncology, medicine.medical_specialty, medicine.medical_treatment, CELL LUNG-CANCER, Population, Peripheral edema, Phases of clinical research, [SDV.CAN]Life Sciences [q-bio]/Cancer, Delta-like protein 3, Rovalpituzumab tesirine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, DRUG, education, ComputingMilieux_MISCELLANEOUS, Chemotherapy, education.field_of_study, Small cell lung cancer, business.industry, Hazard ratio, Confidence interval, 030104 developmental biology, 030220 oncology & carcinogenesis, Topotecan, medicine.symptom, business, medicine.drug
Abstract

INTRODUCTION: DLL3, an atypical Notch ligand, is expressed in SCLC tumors but is not detectable in normal adult tissues. Rovalpituzumab tesirine (Rova-T) is an antibody-drug conjugate containing a DLL3-targeting antibody tethered to a cytotoxic agent pyrrolobenzodiazepine by means of a protease-cleavable linker. The efficacy and safety of Rova-T compared with topotecan as second-line therapy in patients with SCLC expressing high levels of DLL3 (DLL3-high) was evaluated. METHODS: The TAHOE study was an open-label, two-to-one randomized, phase 3 study comparing Rova-T with topotecan as second-line therapy in DLL3-high advanced or metastatic SCLC. Rova-T (0.3 mg/kg) was administered intravenously on day 1 of a 42-day cycle for two cycles, with two additional cycles available to patients who met protocol-defined criteria for continued dosing. Topotecan (1.5 mg/m2) was administered intravenously on days 1 to 5 of a 21-day cycle. The primary end point was overall survival (OS). RESULTS: Patients randomized to Rova-T (n = 296) and topotecan (n = 148) were included in the efficacy analyses. The median age was 64 years, and 77% had the extensive disease at initial diagnosis. The median OS (95% confidence interval) was 6.3 months (5.6-7.3) in the Rova-T arm and 8.6 months (7.7-10.1) in the topotecan arm (hazard ratio, 1.46 [95% confidence interval: 1.17-1.82]). An independent data monitoring committee recommended that enrollment be discontinued because of the shorter OS observed with Rova-T compared with topotecan. Safety profiles for both drugs were consistent with previous reports. CONCLUSIONS: Compared with topotecan, which is the current standard second-line chemotherapy, Rova-T exhibited an inferior OS and higher rates of serosal effusions, photosensitivity reaction, and peripheral edema in patients with SCLC. A considerable unmet therapeutic need remains in this population. ispartof: JOURNAL OF THORACIC ONCOLOGY vol:16 issue:9 pages:1547-1558 ispartof: location:United States status: published

Published
2021
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42. Pneumonias

Author

Mornex, Jean-François, Revel, Didier, Greenland, Timothy, Brune, Jean, and Sperber, Miriam, editor

Published
1990
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43. Blood CD9+ B cell, a biomarker of bronchiolitis obliterans syndrome after lung transplantation

Author

Brosseau, Carole, Danger, Richard, Durand, Maxim, Durand, Eugénie, Foureau, Aurore, Lacoste, Philippe, Tissot, Adrien, Roux, Antoine, Reynaud-Gaubert, Martine, Kessler, Romain, Mussot, Sacha, Dromer, Claire, Brugière, Olivier, Mornex, Jean François, Guillemain, Romain, Claustre, Johanna, Magnan, Antoine, Brouard, Sophie, COLT and SysCLAD Consortia, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Vaincre La Mucoviscidose, ANR-17-CE17-0008,Biket,Cellules B GZMB+, un facteur clé de l'immunité chez l'homme?(2017), and Gasche-Soccal, Paola Marina Alessandra

Subjects
medicine.medical_specialty, medicine.medical_treatment, Basic (laboratory) research/science, basic (laboratory) research, Bronchiolitis obliterans, Bronchiolitis obliterans (BOS), B cell biology, 030230 surgery, Peripheral blood mononuclear cell, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Internal medicine, lung transplantation, medicine, Immunology and Allergy, Lung transplantation, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Pharmacology (medical), pulmonology, science, B cell, ddc:616, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Transplantation, Lung, business.industry, Incidence (epidemiology), Biomarker, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 3. Good health, medicine.anatomical_structure, translational research, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, biomarker, Biomarker (medicine), Translational research/science, Lung transplantation/pulmonology, bronchiolitis obliterans (BOS), business
Abstract

International audience; Bronchiolitis obliterans syndrome is the main limitation for long-term survival after lung transplantation. Some specific B cell populations are associated with long-term graft acceptance. We aimed to monitor the B cell profile during early development of bronchiolitis obliterans syndrome after lung transplantation. The B cell longitudinal profile was analyzed in peripheral blood mononuclear cells from patients with bronchiolitis obliterans syndrome and patients who remained stable over 3 years of follow-up. CD24(hi)CD38(hi) transitional B cells were increased in stable patients only, and reached a peak 24 months after transplantation, whereas they remained unchanged in patients who developed a bronchiolitis obliterans syndrome. These CD24(hi)CD38(hi) transitional B cells specifically secrete IL-10 and express CD9. Thus, patients with a total CD9(+) B cell frequency below 6.6% displayed significantly higher incidence of bronchiolitis obliterans syndrome (AUC = 0.836, PPV = 0.75, NPV = 1). These data are the first to associate IL-10-secreting CD24(hi)CD38(hi) transitional B cells expressing CD9 with better allograft outcome in lung transplant recipients. CD9-expressing B cells appear as a contributor to a favorable environment essential for the maintenance of long-term stable graft function and as a new predictive biomarker of bronchiolitis obliterans syndrome-free survival.

Published
2019
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44. Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension

Author

Boucly, Athénaïs, primary, Savale, Laurent, additional, Jaïs, Xavier, additional, Bauer, Fabrice, additional, Bergot, Emmanuel, additional, Bertoletti, Laurent, additional, Beurnier, Antoine, additional, Bourdin, Arnaud, additional, Bouvaist, Hélène, additional, Bulifon, Sophie, additional, Chabanne, Céline, additional, Chaouat, Ari, additional, Cottin, Vincent, additional, Dauphin, Claire, additional, Degano, Bruno, additional, De Groote, Pascal, additional, Favrolt, Nicolas, additional, Feng, Yuanchao, additional, Horeau-Langlard, Delphine, additional, Jevnikar, Mitja, additional, Jutant, Etienne-Marie, additional, Liang, Zhiying, additional, Magro, Pascal, additional, Mauran, Pierre, additional, Moceri, Pamela, additional, Mornex, Jean-François, additional, Palat, Sylvain, additional, Parent, Florence, additional, Picard, François, additional, Pichon, Jérémie, additional, Poubeau, Patrice, additional, Prévot, Grégoire, additional, Renard, Sébastien, additional, Reynaud-Gaubert, Martine, additional, Riou, Marianne, additional, Roblot, Pascal, additional, Sanchez, Olivier, additional, Seferian, Andrei, additional, Tromeur, Cécile, additional, Weatherald, Jason, additional, Simonneau, Gérald, additional, Montani, David, additional, Humbert, Marc, additional, and Sitbon, Olivier, additional

Published
2021
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46. Prevalence of Anti-Neutrophil Cytoplasmic Antibodies and Associated Vasculitis in COPD Associated With Alpha-1 Antitrypsin Deficiency: An Ancillary Study to a Prospective Study on 180 French Patients

Author

Deshayes, Samuel, Martin Silva, Nicolas, Khoy, Kathy, Mariotte, Delphine, Le Mauff, Brigitte, Mornex, Jean-François, Pison, Christophe, Cuvelier, Antoine, Balduyck, Malika, Pujazon, Marie-Christine, Fournier, Michel, Ait Ilalne, Brahim, Thabut, Gabriel, Mal, Hervé, and Aouba, Achille

Published
2020
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47. Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort

Author

Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, OrphaLung Network, UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), CHU Lille, Rheinisch-Westfälische Technische Hochschule Aachen University (RWTH), Hopital Saint-Louis [AP-HP] (AP-HP), Hôpital Beaujon [AP-HP], Les Hôpitaux Universitaires de Strasbourg (HUS), Hôpital Foch [Suresnes], Université Paris-Saclay, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Cliniques Universitaires Saint-Luc [Bruxelles], Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), École des Hautes Études en Santé Publique [EHESP] (EHESP), Hôpital Avicenne [AP-HP], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Hôpital Nord [CHU - APHM], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Rheinisch-Westfälische Technische Hochschule Aachen (RWTH), Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Université d'Angers (UA)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )

Subjects
Male, medicine.medical_specialty, lung disease, Anemia, medicine.medical_treatment, [SDV]Life Sciences [q-bio], transplant social worker, 030204 cardiovascular system & hematology, Neutropenia, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, Internal medicine, Pulmonary fibrosis, medicine, lung transplantation, Immunology and Allergy, Lung transplantation, Humans, Pharmacology (medical), Telomerase, pulmonology, Retrospective Studies, Transplantation, Lung, business.industry, fibrosis, Retrospective cohort study, Middle Aged, medicine.disease, Idiopathic Pulmonary Fibrosis, practice, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, clinical research, Cohort, Mutation, business
Abstract

Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.

Published
2021
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48. Airway complications in lung transplant recipients with telomere-related interstitial lung disease.

Author

UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, Choi, Bina, Messika, Jonathan, Courtwright, Andrew, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Le Pavec, Jérôme, Quêtant, Sébastien, Froidure, Antoine, Lazor, Romain, Reynaud-Gaubert, Martine, Borgne, Aurélie Le, Houlbracq, Mathilde Phillips, Goldberg, Hilary, El-Chemaly, Souheil, Borie, Raphael, French Group of Lung Transplantation, UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, Choi, Bina, Messika, Jonathan, Courtwright, Andrew, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Le Pavec, Jérôme, Quêtant, Sébastien, Froidure, Antoine, Lazor, Romain, Reynaud-Gaubert, Martine, Borgne, Aurélie Le, Houlbracq, Mathilde Phillips, Goldberg, Hilary, El-Chemaly, Souheil, Borie, Raphael, and French Group of Lung Transplantation

Abstract

Patients with short telomere-related interstitial lung disease (ILD) have worse outcomes after lung transplantation. We hypothesized that post-transplant airway complications, including dehiscence and bronchial stenosis, would be more common in the short telomere ILD lung transplant population. We conducted a multi-institutional (Brigham and Women's Hospital, Groupe de Transplantation de la SPLF) retrospective cohort study of 63 recipients between 2009 and 2019 with ILD and short telomeres, compared to 4359 recipients from the Scientific Registry of Transplant Recipients with ILD and no known telomeropathy. In the short telomere cohort, six recipients (9.5%) developed dehiscence and nine recipients (14.3%) developed stenosis, compared to 60 (1.4%) and 149 (3.4%) in the control, respectively. After adjusting for age, sex, and bilaterality, the presence of short telomeres was associated with higher odds of dehiscence (odds ratio (OR) = 8.24, 95% confidence interval (CI) = 3.34 20.29, p < .001) and stenosis (OR = 4.63, 95% CI 2.21 9.69, p < .001). The association between the presence of short telomeres and post-transplant dehiscence and stenosis suggest that airway complications may be a contributor to increased morbidity and mortality in patients with telomere-related ILD.

Published
2021

49. Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.

Author

UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, OrphaLung Network, UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, and OrphaLung Network

Abstract

Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.

Published
2021

50. Lung transplantation for sarcoidosis:Outcome and prognostic factors

Author

Le Pavec, Jérôme, Valeyre, Dominique, Gazengel, Pierre, Holm, Are M., Schultz, Hans Henrik, Perch, Michael, Le Borgne, Aurélie, Reynaud-Gaubert, Martine, Knoop, Christiane, Godinas, Laurent, Hirschi, Sandrine, Bunel, Vincent, Laporta, Rosalia, Harari, Sergio, Blanchard, Elodie, Magnusson, Jesper M., Tissot, Adrien, Mornex, Jean François, Picard, Clément, Savale, Laurent, Bernaudin, Jean François, Brillet, Pierre Yves, Nunes, Hilario, Humbert, Marc, Fadel, Elie, Gottlieb, Jens, Le Pavec, Jérôme, Valeyre, Dominique, Gazengel, Pierre, Holm, Are M., Schultz, Hans Henrik, Perch, Michael, Le Borgne, Aurélie, Reynaud-Gaubert, Martine, Knoop, Christiane, Godinas, Laurent, Hirschi, Sandrine, Bunel, Vincent, Laporta, Rosalia, Harari, Sergio, Blanchard, Elodie, Magnusson, Jesper M., Tissot, Adrien, Mornex, Jean François, Picard, Clément, Savale, Laurent, Bernaudin, Jean François, Brillet, Pierre Yves, Nunes, Hilario, Humbert, Marc, Fadel, Elie, and Gottlieb, Jens

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.

Published
2021

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