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6. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study

Author

Kanaan, Reem, Carlier, Nicolas, Honoré, Isabelle, Chedevergne, Frédérique, Dreano, Elise, Hatton, Aurélie, Hinzpeter, Alexandre, Pranke, Iwona, Le Clainche-Viala, Laurence, Mayer, Sophie, Corvol, Harriet, Thouvenin, Guillaume, de Miranda, Sandra, Remus, Natascha, Douvry, Benoit, Duthoit, Louise, Perez, Thierry, Le Rouzic, Olivier, Wizla, Nathalie, Bon, Claire, Bui, Stéphanie, Poey, Nora, Stremler, Nathalie, Coltey, Bérengère, Dufeu, Nadine, Lebihan, Jean, Gabsi, Asma, Pouradier, Delphine, Andrejak, Claire, Rames, Cinthia, Dupuy-Grasset, Magali, Languepin, Jeanne, Marguet, Christophe, Pramil, Stéphanie, Arnouat, Baptiste, Fanton, Annlyse, Abely, Michel, Ravoninjatovo, Bruno, Blondé, Aurore, Guillaumot, Anne, Kieffer, Sebastien, Tatopoulos, Aurélie, Nove-Josserand, Raphaële, Ohlmann, Camille, Perrin, Thomas, Reynaud, Quitterie, Llerena, Catherine, Quétant, Sébastien, Valois, Sophie, Dalphin, Marie-Laure, Richaud-Thiriez, Bénédicte, Deneuville, Eric, Chiron, Raphael, Socchi, Floriane, Bihouée, Tiphaine, Mankikian, Julie, Flament, Thomas, Coolen-Allou, Nathalie, Gachelin, Elsa, Périsson, Caroline, Vuillard, Constance, Dupuis, Marion, Alkoussa, Wael, Marchal, Sarah, Leroy, Sylvie, Scalbert, Manuela, Campbell, Karine, Laurans, Muriel, Labbé, Guillaume, Montcouquiol, Sylvie, Priou, Pascaline, de Carli, Paola, Lemonnier, Lydie, Dehillotte, Clémence, Nouvel, Thierry, Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, Ramel, Sophie, Cosson, Laure, Danner-Boucher, Isabelle, Foucaud, Pierre, Roy, Charlotte, Burnet, Espérie, Raynal, Caroline, Audrezet, Marie-Pierre, Da Silva, Jennifer, and Martin, Clémence

Published
2024
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7. Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study

Author

Kasmi, Irena, Drali, Ouardia, Burghart, Sabine, Lakatos-Krepcik, Andrea, Eder, Johannes, Jaksch, Peter, Kainz, Katharina, Kallinger, Margit, Leitner, Alexander, Mozdzen, Marta, Pfleger, Andreas, Renner, Sabine, Stadlinger, Martin, Thir, Christina, Nuriyev, Emil, Boboli, Hedwige, De Wachter, Elke, Dupont, Lieven, Gohy, Sophie, Hanssens, Laurence, Knoop, Christiane, Lammertyn, Elise, Nowé, Vicky, Pirson, Jessica, Thimmesch, Matthieu, Van Braeckel, Eva, Van Hoorenbeeck, Kim, Vanderhelst, Eef, Filho, Eduardo Piacentini, Athanazio, Rodrigo Abensur, Martins, Valéria de Carvalho, Duarte, Marta Cristina, Monte, Luciana de Freitas Velloso, de Fuccio, Marcelo Bicalho, Knabben, Adriana de Siqueira Carvalho, Melloti, Roberta, Meneses, Daniela Gois, Petrova, Guergana, Tješić-Drinković, Duška, Dugac, Andrea Vukić, Bambir, Ivan, Yiallouros, Panayiotis, Bilkova, Alena, Drevinek, Pavel, Macek, Milan, Jr, Olesen, Hanne Vebert, Pressler, Tania, Fouda, Eman Mahmoud, Nasr, Samya, Weldetsadik, Abate Yeshidinber, Al-iede, Montaha, Abdrakhmanov, Olzhas, Corvol, Harriet, Lemonnier-Videau, Lydie, Abely, Michel, Piccini, Carole Bailly, Belleguic, Chantal, Bihouee, Tiphaine, Billon, Yves, Bui, Stéphanie, Camara, Boubou, Cheraud, Marie-Christine, Chiron, Raphael, Duet, Emmanuelle Coirier, Cosson, Laure, Dalphin, Marie-Laure, Boucher, Isabelle Danner, De Miranda, Sandra, Deneuville, Eric, Dubus, Jean-Christophe, Durieu, Isabelle, Epaud, Ralph, Gerardin, Michèle, Grenet, Dominique, Houdouin, Véronique, Huet, Frédéric, Reem, Kanaan, Kessler, Romain, Languepin, Jeanne, Laurans, Muriel, Leroy, Sylvie, Llerena, Cathie, Macey, Julie, Mankikian, Julie, Marguet, Christophe, Martin, Clémence, Mely, Laurent, Mittaine, Marie, Murris-Espin, Marlène, Perisson, Caroline, Prevotat, Anne, Ramel, Sophie, Rames, Cinthia, Reix, Philippe, Revillon, Marine, Reynaud-Gaubert, Martine, Richaud-Thiriez, Bénédicte, Rittie, Jean-Luc, Scalbert-Dujardin, Manuëla, Sermet-Gaudelus, Isabelle, Storni, Véronique, Tatopoulos, Aurélie, Thouvenin, Guillaume, Troussier, Françoise, Weiss, Laurence, Wizla, Nathalie, Behl, Eva-Susanne, Brinkmann, Folke, Claßen, Martin, Graepler-Mainka, Ute, Griese, Matthias, Grübl, Armin, Hammermann, Jutta, Hebestreit, Helge, Heinzmann, Andrea, Herz, Alexander, Kiefer, Alexander, Kinder, Birte, Köster, Holger, Kuhnert, Stefan, Mainz, Jochen, Mayer, Angelika, Naehrig, Susanne, Niehues, Tim, Nüßlein, Thomas, Poplawska, Krystyna, Ringshausen, Felix, Rose, Markus, Rosenecker, Josef, Ruppel, Renate, Scharschinger, Anette, Schropp, Christian, Schwarz, Carsten, Smaczny, Christina, Sommerburg, Olaf, Sutharsan, Sivagurunathan, Stolz, Simone, Thomas, Wolfgang, Wege, Sabine, Welzenbach, Britta, Wollschläger, Bettina, Diamantea, Filia, Hatziagorou, Elpis, Manika, Katerina, Cox, Des, Elnazir, Basil, Fletcher, Godfrey, Gunaratnam, Cedric, McKone, Edward F., Plant, Barry J., Cohen-Cymberknoh, Malena, Gur, Michal, Livnat, Galit, Mei-Zahav, Meir, Amato, Annalisa, Ferrari, Gianluca, Badolato, Raffaele, Poli, Piercarlo, Battistini, Fiorella, Donati, Valentina, Bignamini, Elisabetta, Folino, Anna, Carnovale, Vincenzo, Castellani, Carlo, Casciaro, Rosaria, Cimino, Giuseppe, Cipolli, Marco, Lucca, Francesca, Collura, Mirella, Ficili, Francesca, Daccò, Valeria, Gagliano, Vanessa, Pizzamiglio, Giovanna, Mencarini, Valeria, Palladino, Nicola, Leonardi, Salvatore, Rotolo, Novella, Lucanto, Maria Cristina, Quattromano, Ester, Lucidi, Vincenzina, Majo, Fabio, Alghisi, Federico, Ciciriello, Fabiana, Manca, Antonio, Leonetti, Giuseppina, Maschio, Massimo, Messore, Barbara, Pantano, Stefano, Pisi, Giovanna, Spaggiari, Cinzia, Raia, Valeria, Laezza, Caterina, Ros, Mirco, Salvatore, Donatello, Taccetti, Giovanni, Francalanci, Michela, Vitullo, Pamela, Zolin, Anna, Aleksejeva, Elina, Malakauskas, Kestutis, Misevičiene, Valdone, Charatsi, Anna-Maria, la Barrière, Hélène De, Altenburg, Josje, Bannier, Michiel, Heijerman, Harry, Janssens, Hettie, Koppelman, Gerard, van der Meer, Renske, Merkus, Peter, Nuijsink, Marianne, Terheggen, Suzanne, van der Vaart, Hester, Wesseling, Geert-Jan, de Winter, Karin, Danevska, Ivana Arnaudova, Maretti, Tatjana Jakovska, Fustik, Stojka, Dziecichowicz-Latała, Daria, Wojsyk-Banaszak, Irena, Wozniacki, Lukasz, Amorim, Adelina, Santos, Ana Sofia Araújo, Castanhinha, Susana, Gamboa, Fernanda, Silva, Teresa Reis, Gonçalves, Fabienne, Pereira, Luísa, Ciuca, Ioana, Silva, Sónia, Csilla-Enikö, Szabo, Stan, Iustina, Amelina, Elena, Boitсova, Evgeniya, Chernyavskaya, Anastasia, Gorinova, Yuliya, Krasovskiy, Stanislav, Mukhina, Maria, Sherman, Victoria, Simonova, Olga, Kondratyeva, Elena, Bérešová, Eva, Bližnáková, Nina, Kayserová, Hana, Salobir, Barbara, Šelb, Julij, Krivec, Uroš, Fernandez, Antonio José Aguilar, Fernàndez, Antonio Alvarez, García, Félix Baranda, Aparicio, Marina Blanco, Corullón, Silvia Castillo, Cortell-Aznar, Isidoro, Pérez, Inés, Colomer, Jordi Costa i, Roig, María Cols, Pecellín, Isabel Delgado, Cáceres, Layla Diab, Paredes, Carmen Luna, Gartner, Silvia, Martínez, José Ramón Gutiérrez, Labarga, Inés Herrero, Girón-Moreno, Rosa Maria, Nogueira, Esperanza Jiménez, Ferreiro, Adelaida Lamas, Neyra, Alejandro López, Castro, Enrique Blitz, Galarraga, Laura Moreno, de Vincente, Carlos Martin, Navarro, Silvia Merlos, Nieto-Royo, Rosa, Fuster, Casilda Olveira, Pastor, Maria Dolores, Pérez-Ruiz, Estela, Prados-Sánchez, Concepción, Cancelo, Isabel Ramos, de Valbuena, Marta Ruiz, Asensi, José R. Villa, Santiago, Veronica Sanz, García, Patricia Fernández, Tawfeeq, Reem Mustafa, Banki, Adrienn, Gilljam, Marita, Krantz, Christina, Lindberg, Ulrika, Lindblad, Anders, Clarenbach, Christian, Steinack, Carolin, Hage, René, Schuurmans, Macé, Fischer, Reta, Kusche, Rachel, Rochat, Isabelle, Walter, Anna-Lena, Kamalaporn, Harutai, Hamouda, Samia, Tural, Dilber Ademhan, Ozcelik, Ugur, Asfuroğlu, Pelin, Eyüboğlu, Tuğba Şişmanlar, Aslan, Ayse Tana, Bingöl, Ayşen, Çobanoğlu, Nazan, Ozcan, Gizem, Dogru, Deniz, Gökdemir, Yasemin, KÖSE, Mehmet, Pekcan, Sevgi, Cosgriff, Rebecca, Semenchuk, Julie, Naito, Yumi, Charman, Susan C., Carr, Siobhán B, Cheng, Stephanie Y., Marshall, Bruce C., Faro, Albert, Elbert, Alexander, Gutierrez, Hector H., Goss, Christopher H., Karadag, Bulent, Burgel, Pierre-Régis, Colombo, Carla, Salvatore, Marco, Padoan, Rita, Daneau, Géraldine, Harutyunyan, Satenik, Kashirskaya, Nataliya, Kirwan, Laura, Middleton, Peter G, Ruseckaite, Rasa, de Monestrol, Isabelle, Naehrlich, Lutz, Mondejar-Lopez, Pedro, Jung, Andreas, van Rens, Jacqui, Bakkeheim, Egil, Orenti, Annalisa, Zomer-van Ommen, Domenique, da Silva-Filho, Luiz Vicente RF, Fernandes, Flavia Fonseca, Zampoli, Marco, and Stephenson, Anne L.

Published
2024
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11. French recommendations for the diagnosis and management of lymphangioleiomyomatosis

Author

Cottin, Vincent, Blanchard, Elodie, Kerjouan, Mallorie, Lazor, Romain, Reynaud-Gaubert, Martine, Taille, Camille, Uzunhan, Yurdagül, Wemeau, Lidwine, Andrejak, Claire, Baud, Dany, Bonniaud, Philippe, Brillet, Pierre-Yves, Calender, Alain, Chalabreysse, Lara, Court-Fortune, Isabelle, Desbaillets, Nicolas Pierre, Ferretti, Gilbert, Guillemot, Anne, Hardelin, Laurane, Kambouchner, Marianne, Leclerc, Violette, Lederlin, Mathieu, Malinge, Marie-Claire, Mancel, Alain, Marchand-Adam, Sylvain, Maury, Jean-Michel, Naccache, Jean-Marc, Nasser, Mouhamad, Nunes, Hilario, Pagnoux, Gaële, Prévot, Grégoire, Rousset-Jablonski, Christine, Rouviere, Olivier, Si-Mohamed, Salim, Touraine, Renaud, Traclet, Julie, Turquier, Ségolène, Vagnarelli, Stéphane, and Ahmad, Kaïs

Published
2023
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12. 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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13. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version

Author

Cottin, Vincent, Bonniaud, Philippe, Cadranel, Jacques, Crestani, Bruno, Jouneau, Stéphane, Marchand-Adam, Sylvain, Nunes, Hilario, Wémeau-Stervinou, Lidwine, Bergot, Emmanuel, Blanchard, Elodie, Borie, Raphaël, Bourdin, Arnaud, Chenivesse, Cécile, Clément, Annick, Gomez, Emmanuel, Gondouin, Anne, Hirschi, Sandrine, Lebargy, François, Marquette, Charles-Hugo, Montani, David, Prévot, Grégoire, Quetant, Sébastien, Reynaud-Gaubert, Martine, Salaun, Mathieu, Sanchez, Olivier, Trumbic, Bruno, Berkani, Karim, Brillet, Pierre-Yves, Campana, Marion, Chalabreysse, Lara, Chatté, Gérard, Debieuvre, Didier, Ferretti, Gilbert, Fourrier, Jean-Michel, Just, Nicolas, Kambouchner, Marianne, Legrand, Bertrand, Le Guillou, Frédéric, Lhuillier, Jean-Pierre, Mehdaoui, Anas, Naccache, Jean-Marc, Paganon, Catherine, Rémy-Jardin, Martine, Si-Mohamed, Salim, and Terrioux, Philippe

Published
2023
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15. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study.

Author

Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, and Ramel, Sophie

Subjects
CYSTIC fibrosis, CYSTIC fibrosis transmembrane conductance regulator, LUNGS, CHLORIDES, PERCENTILES
Abstract

Elexacaftor–tezacaftor–ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTR variant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor–tezacaftor–ivacaftor for people with cystic fibrosis without a F508del CFTR variant in France and to determine CFTR variant responsiveness to elexacaftor–tezacaftor–ivacaftor based on the observed clinical response. The French compassionate programme expanded access to elexacaftor–tezacaftor–ivacaftor to people with cystic fibrosis, aged 6 years and older, without a F508del variant, excluding those with two variants previously characterised as non-responsive. Participants at France's 47 cystic fibrosis centres were given a 4–6 week trial of elexacaftor–tezacaftor–ivacaftor and response was determined by a centralised committee based on evolution of clinical data, lung function, and sweat chloride concentration. Responsiveness of individual CFTR variants was derived from observed clinical responses. The first compassionnate programme was launched on May 19, 2022; by March 8, 2024, 516 people with cystic fibrosis had been identified for inclusion in this real-word study: 37 were not included due to the presence of two variants previously characterised as non-responsive to elexacaftor–tezacaftor–ivacaftor, and 479 (229 females [48%] and 250 males [52%]) received elexacaftor–tezacaftor–ivacaftor for 4–6 weeks. Among 443 participants who received no CFTR modulator before elexacaftor–tezacaftor–ivacaftor, 83 had at least one FDA-approved variant, of whom 81 (98%) were responders and continued elexacaftor–tezacaftor–ivacaftor; in responders, mean absolute change in sweat chloride was –44·5 mmol/L (95% CI –39·1 to –49·8) and percentage of predicted FEV 1 (ppFEV 1) was 11·1 percentage points (95% CI 8·4 to 13·7; both comparisons p<0·0001). Among 360 participants with no FDA-approved variant and no previous CFTR modulator, 177 (49%) were responders; in responders, mean absolute change in sweat chloride was –20·5 mmol/L (–17·2 to –23·8) and ppFEV 1 was 13·2 percentage points (11·4 to 15·0; both comparisons p<0·0001). Among 36 participants who were receiving ivacaftor before elexacaftor–tezacaftor–ivacaftor, 32 (89%) continued elexacaftor–tezacaftor–ivacaftor. Of 251 individual CFTR variants, 64 (28 FDA-approved) were classified as responsive or possibly responsive to elexacaftor–tezacaftor–ivacaftor, and 123 (two FDA-approved) as non-responsive or possibly non-responsive to elexacaftor–tezacaftor–ivacaftor. In France, over half of the population with cystic fibrosis without a F508del variant responded to elexacaftor–tezacaftor–ivacaftor, with most responders having no FDA-approved variant. The treatment period was relatively short and further research is warranted to describe the long-term safety and effectiveness of elexacaftor–tezacaftor–ivacaftor in this population. Association Vaincre la Mucoviscidose, Société Française de la Mucoviscidose, and Filière Maladies Rares MUCO-CFTR. [ABSTRACT FROM AUTHOR]

Published
2024
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16. Population pharmacokinetics of mycophenolate in patients treated for interstitial lung disease (EVER‐ILD study).

Author

Xu, Yan‐Min, Ternant, David, Reynaud‐Gaubert, Martine, Bejan‐Angoulvant, Théodora, and Marchand‐Adam, Sylvain

Abstract

Background: Mycophenolate mofetil (MMF) has been used to treat interstitial lung disease (ILD), but mycophenolate (MPA) pharmacokinetics was not reported for this use. This ancillary study of the EVER‐ILD protocol aimed at describing the pharmacokinetic variability of MPA using population modelling in ILD. Methods: Concentrations of MPA were measured during an 8‐h course for 27 ILD patients treated with 1000 mg MMF b.i.d. Absorption, distribution and elimination of MPA were described using population compartment models with first‐order transfer and elimination rate constants, while accounting for both absorption peaks using gamma absorption models. Results: The pharmacokinetics of MPA was best described using a two‐compartment model and two gamma absorption models, model performances of this model were still similar to those of a one gamma absorption model. This pharmacokinetics seemed to be notably influenced by body weight, renal function and inflammatory status. The distribubtion value area under the concentration curve between two administrations of MMF was AUC12 = 52.5 mg.h/L in median (interquartile range: 42.2–58.0 mg.h/L). Conclusion: This is the first study reporting MPA pharmacokinetics in ILD. This pharmacokinetics appears to be similar to other indications and should be further investigated in future studies. [ABSTRACT FROM AUTHOR]

Published
2024
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17. Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial

Author

Naccache, Jean-Marc, Jouneau, Stéphane, Didier, Morgane, Borie, Raphaël, Cachanado, Marine, Bourdin, Arnaud, Reynaud-Gaubert, Martine, Bonniaud, Philippe, Israël-Biet, Dominique, Prévot, Grégoire, Hirschi, Sandrine, Lebargy, François, Marchand-Adam, Sylvain, Bautin, Nathalie, Traclet, Julie, Gomez, Emmanuel, Leroy, Sylvie, Gagnadoux, Frédéric, Rivière, Frédéric, Bergot, Emmanuel, Gondouin, Anne, Blanchard, Elodie, Parrot, Antoine, Blanc, François-Xavier, Chabrol, Alexandre, Dominique, Stéphane, Gibelin, Aude, Tazi, Abdellatif, Berard, Laurence, Brillet, Pierre Yves, Debray, Marie-Pierre, Rousseau, Alexandra, Kerjouan, Mallorie, Freynet, Olivia, Dombret, Marie-Christine, Gamez, Anne-Sophie, Nieves, Ana, Beltramo, Guillaume, Pastré, Jean, Le Borgne-Krams, Aurélie, Dégot, Tristan, Launois, Claire, Plantier, Laurent, Wémeau-Stervinou, Lidwine, Cadranel, Jacques, Chenivesse, Cécile, Valeyre, Dominique, Crestani, Bruno, Cottin, Vincent, Simon, Tabassome, and Nunes, Hilario

Published
2022
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18. Treatment of Idiopathic Pulmonary Fibrosis with Capsule or Tablet Formulations of Pirfenidone in the Real-Life French RaDiCo-ILD Cohort

Author

Cottin, Vincent, Guéguen, Sonia, Nunes, Hilario, Jouneau, Stéphane, Crestani, Bruno, Bonniaud, Philippe, Wemeau, Lidwine, Israël-Biet, Dominique, Reynaud-Gaubert, Martine, Gondouin, Anne, Cadranel, Jacques, Marchand-Adam, Sylvain, Chevereau, Marie, Dufaure-Garé, Isabelle, Amselem, Serge, and Clément, Annick

Published
2022
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19. Impact of Air Pollution and MUC5B Genotype on Survival in Idiopathic Pulmonary Fibrosis

Author

Sesé, Lucile, primary, Borie, Raphaël, additional, Kannengiesser, Caroline, additional, Cottin, Vincent, additional, Israel-Biet, Dominique, additional, Crestani, Bruno, additional, Cadranel, Jacques, additional, Chenivesse, Cécile, additional, Boubaya, Marouane, additional, Valeyre, Dominique, additional, Annesi-Maesano, Isabella, additional, Nunes, Hilario, additional, Bouvry, Diane, additional, Brillet, Pierre Yves, additional, Camus, Philippe, additional, Carton, Zohra, additional, Chabrol, Juliette, additional, Cordier, Jean François, additional, Cracco, Christophe, additional, Delaval, Philippe, additional, Didier, Morgane, additional, Duchemann, Boris, additional, Dudoret, Stéphanie Guillot, additional, Feuillet, Sevrine, additional, Freynet, Olivia, additional, Gagnadoux, Frédéric, additional, Germaud, Patrick, additional, Gindre, Louise, additional, Guetta, André, additional, Haussman, Patrick, additional, Jouneau, Stephane, additional, Kambouchner, Marianne, additional, Khouatra, Chahera, additional, Lacronique, Jacques, additional, Molard, Anita, additional, Picard, Clément, additional, Planes, Carole, additional, Rosental, Paul Andrés, additional, Sanchez, Olivier, additional, Similowski, Thomas, additional, Tazi, Abdellatif, additional, Thiberville, Luc, additional, Uzunhan, Yurdagül, additional, Prévot, Grégoire, additional, Marchand-Adam, Sylvain, additional, Hirschi, Sandrine, additional, Dury, Sandra, additional, Giraud, Violaine, additional, Gondouin, Anne, additional, Bonniaud, Philippe, additional, Traclet, Julie, additional, Juvin, Karine, additional, and Reynaud-Gaubert, Martine, additional

Published
2024
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22. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTRvariant: a real-world study

Author

Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, Ramel, Sophie, Cosson, Laure, Douvry, Benoit, Danner-Boucher, Isabelle, Foucaud, Pierre, Roy, Charlotte, Burnet, Espérie, Raynal, Caroline, Audrezet, Marie-Pierre, Da Silva, Jennifer, Martin, Clémence, Kanaan, Reem, Carlier, Nicolas, Honoré, Isabelle, Chedevergne, Frédérique, Dreano, Elise, Hatton, Aurélie, Hinzpeter, Alexandre, Pranke, Iwona, Le Clainche-Viala, Laurence, Mayer, Sophie, Corvol, Harriet, Thouvenin, Guillaume, de Miranda, Sandra, Remus, Natascha, Douvry, Benoit, Duthoit, Louise, Perez, Thierry, Le Rouzic, Olivier, Wizla, Nathalie, Bon, Claire, Bui, Stéphanie, Poey, Nora, Stremler, Nathalie, Coltey, Bérengère, Dufeu, Nadine, Lebihan, Jean, Gabsi, Asma, Pouradier, Delphine, Andrejak, Claire, Rames, Cinthia, Dupuy-Grasset, Magali, Languepin, Jeanne, Marguet, Christophe, Pramil, Stéphanie, Arnouat, Baptiste, Fanton, Annlyse, Abely, Michel, Ravoninjatovo, Bruno, Blondé, Aurore, Guillaumot, Anne, Kieffer, Sebastien, Tatopoulos, Aurélie, Nove-Josserand, Raphaële, Ohlmann, Camille, Perrin, Thomas, Reynaud, Quitterie, Llerena, Catherine, Quétant, Sébastien, Valois, Sophie, Dalphin, Marie-Laure, Richaud-Thiriez, Bénédicte, Deneuville, Eric, Chiron, Raphael, Socchi, Floriane, Bihouée, Tiphaine, Mankikian, Julie, Flament, Thomas, Coolen-Allou, Nathalie, Gachelin, Elsa, Périsson, Caroline, Vuillard, Constance, Dupuis, Marion, Alkoussa, Wael, Marchal, Sarah, Leroy, Sylvie, Scalbert, Manuela, Campbell, Karine, Laurans, Muriel, Labbé, Guillaume, Montcouquiol, Sylvie, Priou, Pascaline, de Carli, Paola, Lemonnier, Lydie, Dehillotte, Clémence, and Nouvel, Thierry

Abstract

Elexacaftor–tezacaftor–ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTRvariant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor–tezacaftor–ivacaftor for people with cystic fibrosis without a F508del CFTRvariant in France and to determine CFTRvariant responsiveness to elexacaftor–tezacaftor–ivacaftor based on the observed clinical response.

Published
2024
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23. Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases.

Author

Deneuville, Lou, Mageau, Arthur, Debray, Marie Pierre, Sacre, Karim, Costedoat‐Chalumeau, Nathalie, Hachulla, Eric, Uzunhan, Yurdagul, Le Tallec, Erwan, Cadranel, Jacques, Marchand Adam, Sylvain, Montani, David, Rémi‐Jardin, Martine, Reynaud‐Gaubert, Martine, Prevot, Gregoire, Beltramo, Guillaume, Crestani, Bruno, Cottin, Vincent, Borie, Raphael, Ahmad, Kais, and Traclet, Julie

Subjects
INTERSTITIAL lung diseases, SJOGREN'S syndrome, CONNECTIVE tissue diseases, RAYNAUD'S disease, ORGANIZING pneumonia, SYSTEMIC lupus erythematosus, HEART block
Abstract

Background and Objective: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE‐ILD from the OrphaLung and French SLE networks during 2005–2020 aimed to describe the characteristics of patients with SLE‐ILD and analyse factors associated with prognosis. Methods: We analysed data for 89 patients with SLE‐ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27–47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE. Results: Forty two (47.2%) patients were positive for anti‐ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0–14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non‐specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow‐up of 86.5 [39.5–161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5‐year and 10‐year transplantation‐free survival were 96% (92–100) and 87% (78–97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression. Conclusion: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE‐ILD frequently have another connective tissue disease. [ABSTRACT FROM AUTHOR]

Published
2024
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24. Multisystemic Effects of Elexacaftor–Tezacaftor–Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease.

Author

Burgel, Pierre-Régis, Paillasseur, Jean-Louis, Durieu, Isabelle, Reynaud-Gaubert, Martine, Hamidfar, Rebecca, Murris-Espin, Marlène, Danner-Boucher, Isabelle, Chiron, Raphaël, Leroy, Sylvie, Douvry, Benoit, Grenet, Dominique, Mely, Laurent, Ramel, Sophie, Montcouquiol, Sylvie, Burnet, Espérie, Ouaalaya, El Hassane, Sogni, Philippe, Da Silva, Jennifer, and Martin, Clémence

Subjects
LUNGS, CYSTIC fibrosis, LUNG diseases, PULMONARY fibrosis, HEPATIC fibrosis, FORCED expiratory volume
Abstract

Rationale: Limited data exist on the safety and effectiveness of elexacaftor-tezacaftor-ivacaftor (ETI) in people with cystic fibrosis (pwCF) and advanced lung disease. Objectives: To evaluate the effects of ETI in an unselected population of pwCF and advanced lung disease. Methods: A prospective observational study, including all adults aged 18 years and older with percentage predicted forced expiratory volume in 1 second (ppFEV1) ⩽ 40 who initiated ETI from December 2019 to June 2021 in France, was conducted. PwCF were followed until August 8, 2022. Results: ETI was initiated in 434 pwCF with a median ppFEV1 of 30 (interquartile range, 25–35), including 27 with severe cystic fibrosis liver disease and 183 with diabetes. PwCF were followed for a median of 587 (interquartile range, 396–728) days after ETI initiation. Discontinuation of ETI occurred in 12 (2.8%) pwCF and was due mostly to lung transplantation (n = 5) or death (n = 4). Absolute increase in ppFEV1 by a mean of +14.2% (95% confidence interval, 13.1–15.4%) occurred at 1 month and persisted throughout the study. Increase in ppFEV1 in the youngest age quartile was almost twice that of the oldest quartile (P < 0.001); body mass index < 18.5 kg/m2 was found in 38.6% at initiation versus 11.3% at 12 months (P = 0.0001). Increases in serum concentrations of vitamins A and E, but not 25-hydroxy vitamin D3, were observed. Significant reductions in the percentages of pwCF using oxygen therapy, noninvasive ventilation, nutritional support, and inhaled and systemic therapies (including antibiotics) were observed; insulin was discontinued in 12% of patients with diabetes. Conclusions: ETI is safe in pwCF and advanced lung disease, with multisystem pulmonary and extrapulmonary benefits. [ABSTRACT FROM AUTHOR]

Published
2024
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27. Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.

Author

Diesler, Rémi, Legendre, Marie, Si‐Mohamed, Salim, Brillet, Pierre‐Yves, Wemeau, Lidwine, Manali, Effrosyni D., Gagnadoux, Frédéric, Hirschi, Sandrine, Lorillon, Gwenaël, Reynaud‐Gaubert, Martine, Bironneau, Vanessa, Blanchard, Elodie, Bourdin, Arnaud, Dominique, Stéphane, Justet, Aurélien, Macey, Julie, Marchand‐Adam, Sylvain, Morisse‐Pradier, Hélène, Nunes, Hilario, and Papiris, Spyros A.

Subjects
INTERSTITIAL lung diseases, IDIOPATHIC pulmonary fibrosis, PULMONARY fibrosis, ADULTS, COMPUTED tomography, LUNG transplantation
Abstract

Background and Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults. Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed. Results: We included 36 patients (median age: 34 years, 20 males), 22 in the SFTPC group and 14 in the ABCA3 group. Clinical characteristics were similar between groups. Baseline median FVC was 59% ([52–72]) and DLco was 44% ([35–50]). An unclassifiable pattern of fibrosing ILD was the most frequent on chest CT, found in 85% of patients, however with a distinct phenotype with ground‐glass opacities and/or cysts. Nonspecific interstitial pneumonia and usual interstitial pneumonia were the most common histological patterns in the ABCA3 group and in the SFTPC group, respectively. Annually, FVC and DLCO declined by 1.87% and 2.43% in the SFTPC group, respectively, and by 0.72% and 0.95% in the ABCA3 group, respectively (FVC, p = 0.014 and DLCO, p = 0.004 for comparison between groups). Median time to death or lung transplantation was 10 years in the SFTPC group and was not reached at the end of follow‐up in the ABCA3 group. Conclusion: SFTPC and ABCA3‐associated ILD present with a distinct phenotype and prognosis. A radiologic pattern of fibrosing ILD with ground‐glass opacities and/or cysts is frequently found in these rare conditions. [ABSTRACT FROM AUTHOR]

Published
2024
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28. Low Prevalence of HLA-G Antibodies in Lung Transplant Patients Detected using MAIPA-Adapted Protocol

Author

Pedini, Pascal, primary, Hubert, Lucas, additional, Carlini, Federico, additional, Baudey, Jean Baptiste, additional, Tous, Audrey, additional, Jordier, Francois, additional, Basire, Agnès, additional, Bagnis, Claude, additional, Reynaud-Gaubert, Martine, additional, Coiffard, Benjamin, additional, Chiaroni, Jacques, additional, Silvy, Monique, additional, and Picard, Christophe, additional

Published
2023
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29. Liver disease in germline mutations of telomere-related genes: Prevalence, clinical, radiological, pathological features, outcome, and risk factors

Author

Sidali, Sabrina, primary, Borie, Raphaël, additional, de Fontbrune, Flore Sicre, additional, El Husseini, Kinan, additional, Rautou, Pierre-Emmanuel, additional, Lainey, Elodie, additional, Goria, Odile, additional, Crestani, Bruno, additional, Cadrane, Jacques, additional, Cottin, Vincent, additional, Bune, Vincent, additional, Dumortier, Jérôme, additional, Jacquemin, Emmanuel, additional, Reboux, Noémi, additional, Hirschi, Sandrine, additional, Bourdin, Arnaud, additional, Meszaros, Magdalena, additional, Dharancy, Sebastien, additional, Hilaire, Sophie, additional, Mallet, Vincent, additional, Reynaud-Gaubert, Martine, additional, Terriou, Louis, additional, Gottrand, Frédéric, additional, Abou Chahla, Wadih, additional, Khan, Jean-Emmanuel, additional, Carrier, Paul, additional, Saliba, Faouzi, additional, Rubbia-Brandt, Laura, additional, Aubert, John-David, additional, Elkrief, Laure, additional, de Lédinghen, Victor, additional, Abergel, Armand, additional, Olivier, Tournilhac, additional, Houssel, Pauline, additional, Jouneau, Stephane, additional, Wemeau, Lidwine, additional, Bergeron, Anne, additional, Leblanc, Thierry, additional, Ollivier-Hourmand, Isabelle, additional, Nguyen Khac, Eric, additional, Morisse-Pradier, Hélène, additional, Ba, Ibrahima, additional, Boileau, Catherine, additional, Roudot-Thoraval, Françoise, additional, Vilgrain, Valérie, additional, Bureau, Christophe, additional, Nunes, Hilario, additional, Naccache, Jean-Marc, additional, Durand, François, additional, Francoz, Claire, additional, Roulot, Dominique, additional, Valla, Dominique, additional, Paradis, Valérie, additional, Kannengiesser, Caroline, additional, and Plessier, Aurélie, additional

Published
2023
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30. 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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31. Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort

Author

Mabo, Axelle, primary, Borie, Raphael, additional, Wemeau-Stervinou, Lidwine, additional, Uzunhan, Yurdagül, additional, Gomez, Emmanuel, additional, Prevot, Gregoire, additional, Reynaud-Gaubert, Martine, additional, Traclet, Julie, additional, Bergot, Emmanuel, additional, Cadranel, Jacques, additional, Marchand-Adam, Sylvain, additional, Bergeron, Anne, additional, Blanchard, Elodie, additional, Bondue, Benjamin, additional, Bonniaud, Philippe, additional, Bourdin, Arnaud, additional, Burgel, Pierre Regis, additional, Hirschi, Sandrine, additional, Marquette, Charles Hugo, additional, Quétant, Sébastien, additional, Nunes, Hilario, additional, Chenivesse, Cécile, additional, Crestani, Bruno, additional, Guirriec, Yoann, additional, Monnier, Delphine, additional, Ménard, Cédric, additional, Tattevin, Pierre, additional, Cottin, Vincent, additional, Luque Paz, David, additional, and Jouneau, Stéphane, additional

Published
2023
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33. Proteomic analysis of biomarkers associated with long-term survival in systemic sclerosis-related group 3 pulmonary hypertension in the HYPID study

Author

Mismetti, Valentine, primary, Delavenne, Xavier, additional, Montani, David, additional, Bezzeghoud, Souad, additional, Delezay, Olivier, additional, Hodin, Sophie, additional, Launay, David, additional, Marchand-Adam, Sylvain, additional, Nunes, Hilario, additional, Ollier, Edouard, additional, Pastré, Jean, additional, Quetant, Sébastien, additional, Reynaud-Gaubert, Martine, additional, Traclet, Julie, additional, Zeghmar, Sabrina, additional, Bertoletti, Laurent, additional, and Cottin, Vincent, additional

Published
2023
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34. Phenotypic characterization of interstitial lung disease associated with mutations in SFTPC and ABCA3 in adults

Author

Diesler, Rémi, primary, Legendre, Marie, additional, Si-Mohamed, Salim, additional, Brillet, Pierre-Yves, additional, Wemeau, Lidwine, additional, Manali, Effrosyni D, additional, Gagnadoux, Frédéric, additional, Gondouin, Anne, additional, Hirschi, Sandrine, additional, Lorillon, Gwenael, additional, Reynaud-Gaubert, Martine, additional, Bironneau, Vanessa, additional, Blanchard, Elodie, additional, Bourdin, Arnaud, additional, Dominique, Stéphane, additional, Justet, Aurélien, additional, Macey, Julie, additional, Marchand-Adam, Sylvain, additional, Morisse-Pradier, Hélène, additional, Nunes, Hilario, additional, Papiris, Spyros, additional, Traclet, Julie, additional, Crestani, Bruno, additional, Amsellem, Serge, additional, Nathan, Nadia, additional, Borie, Raphaël, additional, and Cottin, Vincent, additional

Published
2023
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35. Real-life multicentric national observational study of the use of nintedanib in systemic sclerosis-associated interstitial lung disease

Author

Koether, Vincent, primary, Launay, David, additional, Reynaud Gaubert, Martine, additional, Prévot, Grégoire, additional, Mouthon, Luc, additional, Borie, Raphael, additional, El Husseini, Kinan, additional, Decker, Paul, additional, Dirou, Stéphanie, additional, Blanchart, Elodie, additional, Leurs, Amélie, additional, Berthier, Sabine, additional, Delbrel, Xavier, additional, Durel, Marie, additional, Agard, Christian, additional, Hachulla, Eric, additional, Nieves, Ana, additional, Aydindag, Denise, additional, Snfmi, Snfmi, additional, Gfrs, Gfrs, additional, Orphalung, Orphalung, additional, Cottin, Vincent, additional, and Uzunhan, Yurdagul, additional

Published
2023
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37. 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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38. Low prevalence of HLA-G antibodies in lung transplant patients detected by MAIPA adapted protocol

Author

PEDINI, PASCAL, primary, HUBERT, Lucas, additional, Carlini, Federico, additional, Baudey, Jean Baptiste, additional, TOUS, Audrey, additional, Jordier, Francois, additional, Basire, Agnes, additional, Bagnis, Claude, additional, Reynaud-Gaubert, Martine, additional, Coiffard, Benjamin, additional, Chiaroni, Jacques, additional, Silvy, Monique, additional, and Picard, Christophe, additional

Published
2023
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39. Proteomic Biomarkers for Survival in Systemic Sclerosis-associated Pulmonary Hypertension

Author

MISMETTI, Valentine, primary, DELAVENNE, Xavier, additional, MONTANI, David, additional, BEZZEGHOUD, Souad, additional, DELEZAY, Olivier, additional, HODIN, Sophie, additional, Launay, David, additional, MARCHAND-ADAM, Sylvain, additional, NUNES, Hilario, additional, Ollier, Edouard, additional, REYNAUD-GAUBERT, Martine, additional, Pastre, Jean, additional, TRACLET, Julie, additional, QUETANT, Sébastien, additional, ZEGHMAR, Sabrina, additional, BERTOLETTI, Laurent, additional, and COTTIN, Vincent, additional

Published
2023
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40. Refractory Pseudomonas aeruginosa Bronchopulmonary Infection After Lung Transplantation for Common Variable Immunodeficiency Despite Maximal Treatment Including IgM/IgA-Enriched Immunoglobulins and Bacteriophage Therapy

Author

Levêque, Manon, primary, Cassir, Nadim, additional, Mathias, Fanny, additional, Fevre, Cindy, additional, Daviet, Florence, additional, Bermudez, Julien, additional, Brioude, Geoffrey, additional, Peyron, Florence, additional, Reynaud-Gaubert, Martine, additional, and Coiffard, Benjamin, additional

Published
2023
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43. 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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44. Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.

Author

Mabo, Axelle, Borie, Raphael, Wemeau-Stervinou, Lidwine, Uzunhan, Yurdagül, Gomez, Emmanuel, Prevot, Gregoire, Reynaud-Gaubert, Martine, Traclet, Julie, Bergot, Emmanuel, Cadranel, Jacques, Marchand-Adam, Sylvain, Bergeron, Anne, Blanchard, Elodie, Bondue, Benjamin, Bonniaud, Philippe, Bourdin, Arnaud, Burgel, Pierre Regis, Hirschi, Sandrine, Marquette, Charles Hugo, and Quétant, Sébastien

Subjects
PULMONARY alveolar proteinosis, LUNG infections
Published
2024
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46. Rituximab and mycophenolate mofetil combination in patients with interstitial lung disease (EVER-ILD): a double-blind, randomised, placebo-controlled trial

Author

Mankikian, Julie, primary, Caille, Agnès, additional, Reynaud-Gaubert, Martine, additional, Agier, Marie-Sara, additional, Bermudez, Julien, additional, Bonniaud, Philippe, additional, Borie, Raphael, additional, Brillet, Pierre-Yves, additional, Cadranel, Jacques, additional, Court-Fortune, Isabelle, additional, Crestani, Bruno, additional, Debray, Marie-Pierre, additional, Gomez, Emmanuel, additional, Gondouin, Anne, additional, Hirschi-Santelmo, Sandrine, additional, Israel-Biet, Dominique, additional, Jouneau, Stéphane, additional, Juvin, Karine, additional, Leger, Julie, additional, Kerjouan, Mallorie, additional, Marquette, Charles-Hugo, additional, Naccache, Jean-Marc, additional, Nunes, Hilario, additional, Plantier, Laurent, additional, Prevot, Grégoire, additional, Quetant, Sébastien, additional, Traclet, Julie, additional, Valentin, Victor, additional, Uzunhan, Yurdagul, additional, Wémeau-Stervinou, Lidwine, additional, Bejan-Angoulvant, Theodora, additional, Cottin, Vincent, additional, and Marchand-Adam, Sylvain, additional

Published
2023
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48. 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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49. Clinical relevance of cell-free DNA quantification and qualification during the first month after lung transplantation

Author

Pedini, Pascal, primary, Coiffard, Benjamin, additional, Cherouat, Nicem, additional, Casas, Sylvia, additional, Fina, Frédéric, additional, Boutonnet, Audrey, additional, Baudey, Jean Baptiste, additional, Aho, Printil, additional, Basire, Agnes, additional, Simon, Sophie, additional, Frassati, Coralie, additional, Chiaroni, Jacques, additional, Reynaud-Gaubert, Martine, additional, and Picard, Christophe, additional

Published
2023
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