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4. 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 M, Cassir N, Mathias F, Fevre C, Daviet F, Bermudez J, Brioude G, Peyron F, Reynaud-Gaubert M, and Coiffard B

Subjects
primary immunodeficiency diseases, lung transplantation, pseudomonas aeruginosa, phage therapy, immunoglobulin therapy, Infectious and parasitic diseases, RC109-216
Abstract

Manon Levêque,1 Nadim Cassir,2 Fanny Mathias,3 Cindy Fevre,4 Florence Daviet,5 Julien Bermudez,1 Geoffrey Brioude,6 Florence Peyron,3 Martine Reynaud-Gaubert,1 Benjamin Coiffard1 1Department of Respiratory Medicine and Lung Transplantation, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 2Department of Infectious Disease, APHM, IHU Méditerranée Infection, Aix-Marseille University, Marseille, France; 3Department of Pharmacy, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 4Research and Development, Pherecydes Pharma, Romainville, France; 5Intensive Care Medicine, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 6Department of Thoracic Surgery and Lung Transplantation, APHM, Aix Marseille University, Hôpital Nord, Marseille, FranceCorrespondence: Benjamin Coiffard, Department of Respiratory Medicine and Lung Transplantation, APHM, CHU Nord, Chemin des Bourrely, Marseille, 13915, France, Tel +33491966133, Email benjamin.coiffard@ap-hm.frAbstract: Recipients transplanted for bronchiectasis in the context of a primary immune deficiency, such as common variable immunodeficiency, are at a high risk of severe infection in post-transplantation leading to poorer long-term outcomes than other transplant indications. In this report, we present a fatal case due to chronic Pseudomonas aeruginosa bronchopulmonary infection in a lung transplant recipient with common variable immunodeficiency despite successful eradication of an extensively drug-resistant (XDR) strain with IgM/IgA-enriched immunoglobulins and bacteriophage therapy. The fatal evolution despite a drastic adaptation of the immunosuppressive regimen and the maximal antibiotic therapy strategy raises the question of the contraindication of lung transplantation in such a context of primary immunodeficiency.Keywords: primary immunodeficiency diseases, lung transplantation, Pseudomonas aeruginosa, phage therapy, immunoglobulin therapy

Published
2023

8. Transplantation pulmonaire en France : actualisation des indications et contre-indications en 2022

Author

Le Pavec, J., Pison, C., Hirschi, S., Bunel, V., Mordant, P., Brugière, O., Le Guen, M., Olland, A., Coiffard, B., Renaud-Picard, B., Tissot, A., Brioude, G., Borie, R., Crestani, B., Deslée, G., Stelianides, S., Mal, H., Schuller, A., Falque, L., Lorillon, G., Tazi, A., Burgel, P.R., Grenet, D., De Miranda, S., Bergeron, A., Launay, D., Cottin, V., Nunes, H., Valeyre, D., Uzunhan, Y., Prévot, G., Sitbon, O., Montani, D., Savale, L., Humbert, M., Fadel, E., Mercier, O., Mornex, J.F., Dauriat, G., and Reynaud-Gaubert, M.

Published
2022
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9. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version intégrale

Author

Cottin, V., Bonniaud, P., Cadranel, J., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunes, H., Wémeau-Stervinou, L., Bergot, E., Blanchard, E., Borie, R., Bourdin, A., Chenivesse, C., Clément, A., Gomez, E., Gondouin, A., Hirschi, S., Lebargy, F., Marquette, C.-H., Montani, D., Prévot, G., Quetant, S., Reynaud-Gaubert, M., Salaun, M., Sanchez, O., Trumbic, B., Berkani, K., Brillet, P.-Y., Campana, M., Chalabreysse, L., Chatté, G., Debieuvre, D., Ferretti, G., Fourrier, J.-M., Just, N., Kambouchner, M., Legrand, B., Le Guillou, F., Lhuillier, J.-P., Mehdaoui, A., Naccache, J.-M., Paganon, C., Rémy-Jardin, M., Si-Mohamed, S., and Terrioux, P.

Published
2022
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10. Atteinte pulmonaire du déficit en alpha-1 antitrypsine. Recommandations pratiques pour le diagnostic et la prise en charge

Author

Mornex, J.-F., Balduyck, M., Bouchecareilh, M., Cuvelier, A., Epaud, R., Kerjouan, M., Le Rouzic, O., Pison, C., Plantier, L., Pujazon, M.-C., Reynaud-Gaubert, M., Toutain, A., Trumbic, B., Willemin, M.-C., Zysman, M., Brun, O., Campana, M., Chabot, F., Chamouard, V., Dechomet, M., Fauve, J., Girerd, B., Gnakamene, C., Lefrançois, S., Lombard, J.-N., Maitre, B., Maynié-François, C., Moerman, A., Payancé, A., Reix, P., Revel, D., Revel, M.-P., Schuers, M., Terrioux, P., Theron, D., Willersinn, F., Cottin, V., and Mal, H.

Published
2022
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11. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version courte

Author

Cottin, V., Bonniaud, P., Cadranel, J., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunes, H., Wémeau-Stervinou, L., Bergot, E., Blanchard, E., Borie, R., Bourdin, A., Chenivesse, C., Clément, A., Gomez, E., Gondouin, A., Hirschi, S., Lebargy, F., Marquette, C.-H., Montani, D., Prévot, G., Quetant, S., Reynaud-Gaubert, M., Salaun, M., Sanchez, O., Trumbic, B., Berkani, K., Brillet, P.-Y., Campana, M., Chalabreysse, L., Chatté, G., Debieuvre, D., Ferretti, G., Fourrier, J.-M., Just, N., Kambouchner, M., Legrand, B., Le Guillou, F., Lhuillier, J.-P., Mehdaoui, A., Naccache, J.-M., Paganon, C., Rémy-Jardin, M., Si-Mohamed, S., and Terrioux, P.

Published
2022
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14. Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial

Author

Abe, S., Aburto, M., Acosta, O., Andrews, C., Antin-Ozerkis, D., Arce, G., Arias, M., Avdeev, S., Barczyk, A., Bascom, R., Bazdyrev, E., Beirne, P., Belloli, E., Bergna, M.A., Bergot, E., Bhatt, N., Blaas, S., Bondue, B., Bonella, F., Britt, E., Buch, K., Burk, J., Cai, H., Cantin, A., Castillo Villegas, D.M., Cazaux, A., Cerri, S., Chaaban, S., Chaudhuri, N., Cottin, V., Crestani, B., Criner, G., Dahlqvist, C., Danoff, S., Dematte D'Amico, J., Dilling, D., Elias, P., Ettinger, N., Falk, J., Fernández Pérez, E.R., Gamez-Dubuis, A., Giessel, G., Gifford, A., Glassberg, M., Glazer, C., Golden, J., Gómez Carrera, L., Guiot, J., Hallowell, R., Hayashi, H., Hetzel, J., Hirani, N., Homik, L., Hope-Gill, B., Hotchkin, D., Ichikado, K., Ilkovich, M., Inoue, Y., Izumi, S., Jassem, E., Jones, L., Jouneau, S., Kaner, R., Kang, J., Kawamura, T., Kessler, R., Kim, Y., Kishi, K., Kitamura, H., Kolb, M., Kondoh, Y., Kono, C., Koschel, D., Kreuter, M., Kulkarni, T., Kus, J., Lebargy, F., León Jiménez, A., Luo, Q., Mageto, Y., Maher, T.M., Makino, S., Marchand-Adam, S., Marquette, C., Martinez, R., Martínez, M., Maturana Rozas, R., Miyazaki, Y., Moiseev, S., Molina-Molina, M., Morrison, L., Morrow, L., Moua, T., Nambiar, A., Nishioka, Y., Nunes, H., Okamoto, M., Oldham, J., Otaola, M., Padilla, M., Park, J.S., Patel, N., Pesci, A., Piotrowski, W., Pitts, L., Poonyagariyagorn, H., Prasse, A., Quadrelli, S., Randerath, W., Refini, R., Reynaud-Gaubert, M., Riviere, F., Rodríguez Portal, J.A., Rosas, I., Rossman, M., Safdar, Z., Saito, T., Sakamoto, N., Salinas Fénero, M., Sauleda, J., Schmidt, S., Scholand, M.B., Schwartz, M., Shapera, S., Shlobin, O., Sigal, B., Silva Orellana, A., Skowasch, D., Song, J.W., Stieglitz, S., Stone, H., Strek, M., Suda, T., Sugiura, H., Takahashi, H., Takaya, H., Takeuchi, T., Thavarajah, K., Tolle, L., Tomassetti, S., Tomii, K., Valenzuela, C., Vancheri, C., Varone, F., Veeraraghavan, S., Villar, A., Weigt, S., Wemeau, L., Wuyts, W., Xu, Z., Yakusevich, V., Yamada, Y., Yamauchi, H., Ziora, D., Wells, Athol U, Flaherty, Kevin R, Brown, Kevin K, Inoue, Yoshikazu, Devaraj, Anand, Richeldi, Luca, Moua, Teng, Crestani, Bruno, Wuyts, Wim A, Stowasser, Susanne, Quaresma, Manuel, Goeldner, Rainer-Georg, Schlenker-Herceg, Rozsa, and Kolb, Martin

Published
2020
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15. Cohorte RADICO-ILD2 (Projet FIFA) : atteintes asymptomatiques chez les apparentés de porteurs de variants de gènes liés aux télomères

Author

Guerin, C., primary, Kannengiesser, C., additional, Bramki, K., additional, Jouneau, S., additional, Prevot, G., additional, Marchand-Adam, S., additional, Dominique, S., additional, Andréjak, C., additional, Wemeau, L., additional, Crestani, B., additional, Lainey, E., additional, Sicre De Fontbrune, F., additional, Plessier, A., additional, Dubus, J.C., additional, Clement, A., additional, Nunes, H., additional, Valeyre, D., additional, Nathan, N., additional, Cadranel, J., additional, Israel Biet, D., additional, Reynaud-Gaubert, M., additional, Bonniaud, P., additional, Dufaure-Gare, I., additional, Plouvier, L., additional, Nasri, I., additional, Lassus, A., additional, Ba, I., additional, Cottin, V., additional, Gueguen, S., additional, and Borie, R., additional

Published
2024
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16. Proteomic biomarkers for survival in systemic sclerosis-associated pulmonary hypertension

Author

Mismetti, V., primary, Delavenne, X., additional, Montani, D., additional, Bezzeghoud, S., additional, Delezay, O., additional, Hodin, S., additional, Launay, D., additional, Marchand-Adam, S., additional, Nunes, H., additional, Ollier, E., additional, Reynaud-Gaubert, M., additional, Pastre, J., additional, Traclet, J., additional, Turquier, S., additional, Quetant, S., additional, Zeghmar, S., additional, Bertoletti, L., additional, and Cottin, V., additional

Published
2024
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17. Rituximab et Mycophénolate Mofétil dans les pneumopathies interstitielles diffuses (EVER-ILD) : résultats à 1 an d’un essai randomisé contrôlé

Author

Mansy, L., primary, Caille, A., additional, Reynaud-Gaubert, M., additional, Bonniaud, P., additional, Borie, R., additional, Cadranel, J., additional, Court-Fortune, I., additional, Crestani, B., additional, Gomez, E., additional, Gondouin, A., additional, Hirschi-Santelmo, S., additional, Israel-Biet, D., additional, Jouneau, S., additional, Marquette, C.H., additional, Naccache, J.M., additional, Nunes, H., additional, Plantier, L., additional, Prevot, G., additional, Quetant, S., additional, Traclet, J., additional, Wémeau-Stervinou, L., additional, Cottin, V., additional, and Marchand-Adam, S., additional

Published
2024
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18. Plasma Acute Phase Proteins as Predictors of Chronic Lung Allograft Dysfunction in Lung Transplant Recipients

Author

Janciauskiene S, Royer PJ, Fuge J, Wrenger S, Chorostowska-Wynimko J, Falk C, Welte T, Reynaud-Gaubert M, Roux A, Tissot A, and Magnan A

Subjects
acute phase proteins, transplantation, allograft dysfunction, Pathology, RB1-214, Therapeutics. Pharmacology, RM1-950
Abstract

Sabina Janciauskiene,1,2,* Pierre-Joseph Royer,3,* Jan Fuge,1 Sabine Wrenger,1 Joanna Chorostowska-Wynimko,2 Christine Falk,4,5 Tobias Welte,1 Martine Reynaud-Gaubert,6 Antoine Roux,7– 9 Adrien Tissot,3 Antoine Magnan3 1Department of Pulmonary and Infectious Diseases, BREATH German Center for Lung Research (DZL) Hannover University School, Hannover, Germany; 2Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, Warsaw, Poland; 3CHU de Nantes, Centre National De Référence Mucoviscidose Nantes-Roscoff, Nantes, France; 4Institute of Transplant Immunology, Hannover Medical School, Hannover, Germany; 5German Center for Infection Research DZIF Hannover Braunschweig Site, TTU-IICH, Hannover, Germany; 6Department ofPulmonary Diseases and Lung Transplantation, CHU Nord de Marseille; IHU - Méditerranée Infection, Aix Marseille Université, Marseille, France; 7Hôpital Foch, Suresnes, France; 8Université Versailles Saint-Quentin- en-Yvelines, Versailles, France; 9 l’Institut du Thorax, Université de Nantes, Nantes, France*These authors contributed equally to this workCorrespondence: Sabina Janciauskiene Department of Pulmonary and InfectiousDiseases, Hannover Medical School, Feodor-Lynen Str. 23, Hannover 30625, GermanyTel +49-511-532-7297Email Janciauskiene.sabina@mh-hannover.deAbstract: Cumulating reports suggest that acute phase proteins (APPs) have diagnostic and prognostic value in different clinical conditions. Among others, APPs are proposed to serve as markers that help to control the outcome of transplant recipients. Here, we questioned whether plasma concentrations of APPs mirror the development of chronic lung allograft dysfunction (CLAD). We performed blinded analysis of serial plasma samples retrospectively collected from 35 lung transplanted patients, of whom 25 developed CLAD and 10 remained stable during the follow-up period of 3 to 4.5 years. Albumin (ALB), alpha1-antitrypsin (AAT), high sensitivity C-reactive protein (CRPH), antithrombin-3 (AT3), ceruloplasmin (CER), and alpha2-macroglobulin (A2MG) were measured by the nephelometric method. We found that within the first six months post-transplantation, levels of A2MG, CER and AAT were higher in stable patients relative to those who later developed CLAD. Moreover, in stable patient’s plasma CRPH levels decreased during the follow-up period whereas opposite, in those developing CLAD, the CRPH gradually increased. The ALB levels became significantly lower at the end of the follow-up period in CLAD relative to a stable group. A logistic regression model based on A2MG, CER and AT3 at cut-offs levels of ≥ 175.5 mg/dL, ≥ 37.8 mg/dL and ≥ 27.35 mg/dL enabled to discriminate between stable and CLAD patients with a sensitivity of 87.5%, 100% and 62.5%, and specificity of 65.9%, 72.7% and 79.5%, respectively. We identified A2MG (below  175.5 mg/dL) as an independent predictor of CLAD (hazard ratio 11.5, 95% CI (1.5– 91.3), p< 0.021). Our findings suggest that profiles of certain APPs may help to predict the development of lung dysfunction at the very early stages after transplantation.Keywords: acute phase proteins, transplantation, allograft dysfunction

Published
2020

22. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2017 update. Full-length version

Author

Cottin, V., Crestani, B., Cadranel, J., Cordier, J.-F., Marchand-Adam, S., Prévot, G., Wallaert, B., Bergot, E., Camus, P., Dalphin, J.-C., Dromer, C., Gomez, E., Israel-Biet, D., Jouneau, S., Kessler, R., Marquette, C.-H., Reynaud-Gaubert, M., Aguilaniu, B., Bonnet, D., Carré, P., Danel, C., Faivre, J.-B., Ferretti, G., Just, N., Lebargy, F., Philippe, B., Terrioux, P., Thivolet-Béjui, F., Trumbic, B., and Valeyre, D.

Published
2017
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23. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2017 update. Short-length version

Author

Cottin, V., Crestani, B., Cadranel, J., Cordier, J.-F., Marchand-Adam, S., Prévot, G., Wallaert, B., Bergot, E., Camus, P., Dalphin, J.-C., Dromer, C., Gomez, E., Israel-Biet, D., Jouneau, S., Kessler, R., Marquette, C.-H., Reynaud-Gaubert, M., Aguilaniu, B., Bonnet, D., Carré, P., Danel, C., Faivre, J.-B., Ferretti, G., Just, N., Lebargy, F., Philippe, B., Terrioux, P., Thivolet-Béjui, F., Trumbic, B., and Valeyre, D.

Published
2017
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24. T Cells Promote Bronchial Epithelial Cell Secretion of Matrix Metalloproteinase-9 via a C-C Chemokine Receptor Type 2 Pathway: Implications for Chronic Lung Allograft Dysfunction

Author

Pain, M., Royer, P.-J., Loy, J., Girardeau, A., Tissot, A., Lacoste, P., Roux, A., Reynaud-Gaubert, M., Kessler, R., Mussot, S., Dromer, C., Brugière, O., Mornex, J.-F., Guillemain, R., Dahan, M., Knoop, C., Botturi, K., Pison, C., Danger, R., Brouard, S., and Magnan, A.

Published
2017
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25. (1224) Peripheral Vesicular-Bound Hla-g as Predictor of Graft Tolerance after Lung Transplantation

Author

Brugiere, O., primary, Dreyfuss, D., additional, Guilet, R., additional, Hirschi, S., additional, Renaud-Picard, B., additional, Reynaud-Gaubert, M., additional, Nieves, A., additional, Bunel, V., additional, Messika, J., additional, Demant, X., additional, Jérôme, L., additional, Dauriat, G., additional, Saint-Raymond, C., additional, Falque, L., additional, Mornex, J., additional, Tissot, A., additional, Foureau, A., additional, Leborgne-Krams, A., additional, Boussaud, V., additional, MAgnan, A., additional, Picard, C., additional, Roux, A., additional, Carosella, E. Edgardo, additional, Vallée, A., additional, Freiss, R. Rouas, additional, and MAoult, J. Le, additional

Published
2023
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27. Ziritaxestat, a novel autotaxin inhibitor, and lung function in idiopathic pulmonary fibrosis

Author

Maher, TM, Ford, P, Brown, KK, Costabel, U, Cottin, V, Danoff, SK, Groenveld, I, Helmer, E, Jenkins, RG, Milner, J, Molenberghs, G, Penninckx, B, Randall, MJ, Van Den Blink, B, Fieuw, A, Vandenrijn, C, Rocak, S, Seghers, I, Shao, L, Taneja, A, Jentsch, G, Watkins, TR, Wuyts, WA, Kreuter, M, Verbruggen, N, Prasad, N, Wijsenbeek, MS, Chambers, D, Chia, M, Corte, T, Glaspole, I, Goh, N, Holmes, M, Malouf, M, Thien, F, Veitch, E, Bondue, B, Dahlqvist, C, Froidure, A, Slabbynck, H, Wuyts, W, Cartagena Salinas, C, Feijoó Seoane, R, Martínez, V, Maturana, R, Pavie Gallegos, J, Rosenblut, A, Silva, R, Undurraga Pereira, A, Doubkova, M, Pauk, N, Plackova, M, Sterclova, M, Bendstrup, E, Shaker, SB, Titlestad, I, Budweiser, S, Grohé, C, Koschel, D, Prasse, A, Weber, M, Wirtz, H, Antoniou, K, Daniil, Z, Gaga, M, Papakosta, D, Izumi, S, Okamoto, M, Guerreros Benavides, A, Iberico Barrera, C, Peña Villalobos, AM, Campo Ezquibela, A, Cifrian Martinez, JM, Fernandez Fabrellas, E, Leiro, V, Molina-Molina, M, Nieto Barbero, A, Sellares Torres, J, Valenzuela, C, Cheng, S-L, Kuo, P-H, Lee, K-Y, Sheu, C-C, Gunen, H, Mogulkoc Bishop, N, Nayci, S, Adamali, H, Bianchi, S, Chaudhuri, N, Gibbons, M, Hart, S, Molyneaux, P, Parfrey, H, Saini, G, Spencer, LG, Wiscombe, S, Antin-Ozerkis, D, Bascom, R, Belperio, J, Britt, E, Fitzgerald, J, Gomez Manjarres, D, Gotfried, M, Gupta, N, Hotchkin, D, Kaye, M, Kreider, M, Kureishy, S, Lacamera, P, Lancaster, L, Lasky, J, Lorch, D, Mannem, H, Morrow, L, Moua, T, Nambiar, A, Raghu, G, Raj, R, Ramaswamy, M, Reddy, R, Russell, T, Scholand, MB, Shea, B, Suliman, S, Swigris, J, Thavarajah, K, Tolle, L, Tomic, R, Warshoff, N, Wesselius, L, Yung, G, Bergna, M, De Salvo, M, Fernandez Acquier, M, Rodriguez, A, Saez Scherbovsky, P, Assayag, D, Dhar, A, Khalil, N, Morisset, J, Provencher, S, Ryerson, C, Shapera, S, Bourdin, A, Crestani, B, Lebargy, F, Reynaud-Gaubert, M, Bonella, FT, Claussen, M, Hammerl, P, Karagiannidis, C, Keller, C, Randerath, W, Stubbe, B, Csánky, E, Medgyasszay, B, Muller, V, Adir, Y, Bar-Shai, A, Berkman, N, Fink, G, Kramer, M, Shitrit, D, Bargagli, E, Gasparini, S, Harari, S, Ravaglia, C, Richeldi, L, Vancheri, C, Ebina, M, Fujita, M, Ichikado, K, Inoue, Y, Ishikawa, N, Kato, M, Kawamura, T, Kondoh, Y, Nishioka, Y, Ogura, T, Owan, I, Saito, T, Sakamoto, N, Sakamoto, K, Shirai, M, Suda, T, Tomii, K, Chung, MP, Jeong, SH, Park, CS, Park, JS, Song, JW, Uh, S-T, Chavarria Martinez, U, Montano Gonzalez, E, Ramirez, A, Selman Lama, ME, Bresser, P, Kramer, H, Mostard, R, Nossent, E, Veltkamp, M, Wijsenbeek, M, Beckert, L, Chang, CL, Veale, A, Wilsher, M, Bednarek, M, Gasior, G, Jasieniak-Pinis, G, Jassem, E, Mroz, R, Piotrowski, W, Abdullah, I, Ambaram, A, Irusen, E, Van der Linden, M, Van Zyl-Smit, R, Williams, P, Allen, J, Averill, F, Belloli, E, Brown, A, Case, A, Chaudhary, S, Criner, G, DeBoer, K, Dilling, D, Dorf, J, Enelow, R, Ettinger, N, Feldman, J, Gibson, K, Golden, J, Hamblin, M, Hunninghake, G, Karunakara, R, Kim, H, Luckhardt, T, Menon, P, Morrison, L, Oldham, J, Patel, N, Schmidt, S, Strek, M, Summer, R, Sussman, R, Tita, J, Veeraraghavan, S, Whelan, T, and Zibrak, J

Abstract

Importance There is a major need for effective, well-tolerated treatments for idiopathic pulmonary fibrosis (IPF). Objective To assess the efficacy and safety of the autotaxin inhibitor ziritaxestat in patients with IPF. Design, Setting, and Participants The 2 identically designed, phase 3, randomized clinical trials, ISABELA 1 and ISABELA 2, were conducted in Africa, Asia-Pacific region, Europe, Latin America, the Middle East, and North America (26 countries). A total of 1306 patients with IPF were randomized (525 patients at 106 sites in ISABELA 1 and 781 patients at 121 sites in ISABELA 2). Enrollment began in November 2018 in both trials and follow-up was completed early due to study termination on April 12, 2021, for ISABELA 1 and on March 30, 2021, for ISABELA 2. Interventions Patients were randomized 1:1:1 to receive 600 mg of oral ziritaxestat, 200 mg of ziritaxestat, or placebo once daily in addition to local standard of care (pirfenidone, nintedanib, or neither) for at least 52 weeks. Main Outcomes and Measures The primary outcome was the annual rate of decline for forced vital capacity (FVC) at week 52. The key secondary outcomes were disease progression, time to first respiratory-related hospitalization, and change from baseline in St George’s Respiratory Questionnaire total score (range, 0 to 100; higher scores indicate poorer health-related quality of life). Results At the time of study termination, 525 patients were randomized in ISABELA 1 and 781 patients in ISABELA 2 (mean age: 70.0 [SD, 7.2] years in ISABELA 1 and 69.8 [SD, 7.1] years in ISABELA 2; male: 82.4% and 81.2%, respectively). The trials were terminated early after an independent data and safety monitoring committee concluded that the benefit to risk profile of ziritaxestat no longer supported their continuation. Ziritaxestat did not improve the annual rate of FVC decline vs placebo in either study. In ISABELA 1, the least-squares mean annual rate of FVC decline was –124.6 mL (95% CI, −178.0 to −71.2 mL) with 600 mg of ziritaxestat vs –147.3 mL (95% CI, −199.8 to −94.7 mL) with placebo (between-group difference, 22.7 mL [95% CI, −52.3 to 97.6 mL]), and –173.9 mL (95% CI, −225.7 to −122.2 mL) with 200 mg of ziritaxestat (between-group difference vs placebo, −26.7 mL [95% CI, −100.5 to 47.1 mL]). In ISABELA 2, the least-squares mean annual rate of FVC decline was –173.8 mL (95% CI, −209.2 to −138.4 mL) with 600 mg of ziritaxestat vs –176.6 mL (95% CI, −211.4 to −141.8 mL) with placebo (between-group difference, 2.8 mL [95% CI, −46.9 to 52.4 mL]) and –174.9 mL (95% CI, −209.5 to −140.2 mL) with 200 mg of ziritaxestat (between-group difference vs placebo, 1.7 mL [95% CI, −47.4 to 50.8 mL]). There was no benefit with ziritaxestat vs placebo for the key secondary outcomes. In ISABELA 1, all-cause mortality was 8.0% with 600 mg of ziritaxestat, 4.6% with 200 mg of ziritaxestat, and 6.3% with placebo; in ISABELA 2, it was 9.3% with 600 mg of ziritaxestat, 8.5% with 200 mg of ziritaxestat, and 4.7% with placebo. Conclusions and Relevance Ziritaxestat did not improve clinical outcomes compared with placebo in patients with IPF receiving standard of care treatment with pirfenidone or nintedanib or in those not receiving standard of care treatment. Trial Registration ClinicalTrials.gov Identifiers: NCT03711162 and NCT03733444

Published
2023

29. Altered pIgR/IgA mucosal immunity in bronchiolitis obliterans syndrome

Author

Carlier, F., primary, Pretolani, M., additional, Detry, B., additional, Heddebaut, N., additional, Planté-Bordeneuve, T., additional, Longchampt, E., additional, Falque, L., additional, Reynaud-Gaubert, M., additional, Hirschi, S., additional, Demant, X., additional, Mornex, J., additional, Tissot, A., additional, Le Pavec, J., additional, Messika, J., additional, Foureau, A., additional, Vallée, A., additional, Pilette, C., additional, and Brugière, O., additional

Published
2023
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30. Caractérisation phénotypique des pneumopathies interstitielles diffuses liées à des mutations de SFTPC et d’ABCA3 chez l’adulte

Author

Diesler, R., primary, Nathan, N., additional, Legendre, M., additional, Amsellem, S., additional, Borie, R., additional, Bourdin, A., additional, Nunes, H., additional, Wemeau, L., additional, Gondouin, A., additional, Dominique, S., additional, Morisse Pradier, H., additional, Blanchard, E., additional, Macey, J., additional, Bironneau, V., additional, Gagnadoux, F., additional, Manali, E., additional, Papiris, S., additional, Hirschi, S., additional, Marchand-Adam, S., additional, Justet, A., additional, Reynaud-Gaubert, M., additional, Lorillon, G., additional, Brillet, P.Y., additional, Si-Mohamed, S., additional, and Cottin, V., additional

Published
2023
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31. Implication de la longueur des télomères du donneur et du receveur dans la survenue d’une dysfonction chronique du greffon après transplantation d’organe

Author

Sandot, A., primary, Ba, I., additional, Borie, R., additional, Kessler, R., additional, Reynaud Gaubert, M., additional, Demant, X., additional, Falque, L., additional, Roux, A., additional, Le Pavec, J., additional, Tissot, A., additional, Mornex, J.-F., additional, Leborgne, A., additional, Knoop, C., additional, Bunel-Gourdy, V., additional, Boussaud, V., additional, Mordant, P., additional, Kannengiesser, C., additional, and Messika, J., additional

Published
2023
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32. Implication du polymorphisme MUC5B du donneur et du receveur dans la dysfonction chronique du greffon pulmonaire

Author

Sandot, A., primary, Ba, I., additional, Borie, R., additional, Kessler, R., additional, Reynaud Gaubert, M., additional, Demant, X., additional, Falque, L., additional, Roux, A., additional, Le Pavec, J., additional, Tissot, A., additional, Mornex, J.F., additional, Leborgne, A., additional, Knoop, C., additional, Bunel-Gourdy, V., additional, Boussaud, V., additional, Mordant, P., additional, Kannengiesser, C., additional, and Messika, J., additional

Published
2023
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33. Epidémiologie des pneumopathies interstitielles diffuses en Guadeloupe : étude rétrospective 2013–2019

Author

Neveu, S., primary, Chaptal, M., additional, Rossigneux, E., additional, Philbert, S., additional, Gallois, J., additional, Etienne, N., additional, Testaert, H., additional, Claudeon, J., additional, Reynaud-Gaubert, M., additional, Boulard, F., additional, Receveur, M., additional, Carles, M., additional, Curlier, E., additional, Elourimi, G., additional, Ponce, E., additional, Bailletx, G., additional, Cadelis, G., additional, and Raherison-Semjen, C., additional

Published
2023
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35. Étude nationale multicentrique sur l’utilisation du nintedanib en vie réelle dans la pneumopathie interstitielle diffuse liée à la sclérodermie systémique

Author

Koether, V., primary, Launay, D., additional, Reynaud-Gaubert, M., additional, Prevot, G., additional, Mouthon, L., additional, Borie, R., additional, El Husseini, K., additional, Decker, P., additional, Dirou, S., additional, Blanchart, E., additional, Leurs, A., additional, Berthier, S., additional, Delbrel, X., additional, Durel, M., additional, Agard, C., additional, Nieves, A., additional, Hachulla, E., additional, Aydindag, D., additional, Cottin, V., additional, and Uzunhan, Y., additional

Published
2022
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38. Altered pIgR/IgA mucosal immunity in bronchiolitis obliterans syndrome

Author

Carlier, F, Pretolani, M, Detry, B, Heddebaut, N, Planté-Bordeneuve, T, Longchampt, E, Falque, L, Reynaud-Gaubert, M, Hirschi, S, Demant, X, Mornex, J, Tissot, A, Le Pavec, J, Messika, J, Foureau, A, Vallée, A, Pilette, C, Brugière, O, The Colt Consortium, and UCL - (MGD) Service de pneumologie

Abstract

Aims: Long-term survival after lung transplantation (LT) is hampered by the occurrence of chronic lung allograft dysfunction (CLAD), manifesting as bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS). CLAD is triggered by several factors, e.g. recurrent infections. As immunoglobulin (Ig) A is crucial to ensure mucosal immunity and limit airway microbial load, we explored whether IgA and its epithelial receptor, the polymeric Ig receptor (pIgR) are impaired in BOS. Methods: Bronchoalveolar lavages (BAL, n=120) from LT recipients included in the Cohort for Lung Transplantation were collected at pre-defined timepoints prior to the diagnosis of functional stability (BOS-free, n=30) or BOS (pre-BOS, n=30), and assessed for secretory (S)-IgA. Bronchiolar epithelium pIgR expression was quantified in transbronchial biopsies from BOS-free (n=20), pre-BOS (n=19) and BOS LT recipients (n=12), as well as in end-stage BOS explants (n=15). Results: S-IgA levels were reduced in BAL from pre-BOS LT recipients versus BOS-free (16.1 vs 33.4 µg/ml, p

Published
2022
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39. [French practical guidelines for the diagnosis and management of IPF - 2021 update, full version]

Author

Cottin, V, Bonniaud, P, Cadranel, J, Crestani, B, Jouneau, S, Marchand-Adam, S, Nunes, H, Wémeau-Stervinou, L, Bergot, E, Blanchard, E, Borie, R, Bourdin, A, Chenivesse, C, Clément, Annick, Gomez-Quiroz, Luis E, Gondouin, A, Hirschi, S, Lebargy, F, Marquette, C.-H., Montani, D, Prévot, G, Quetant, S, Reynaud-Gaubert, M, Salaün, M, Sanchez, O, Trumbic, B, Berkani, K, Brillet, P.-Y., Campana, M, Chalabreysse, L, Chatté, G, Debieuvre, D, Ferretti, G, Fourrier, J.-M., Just, N, Kambouchner, M, Legrand, B, Le Guillou, F, Lhuillier, J.-P., Mehdaoui, A., Naccache, J.-M., Paganon, C, Rémy-Jardin, M, Si-Mohamed, S., Terrioux, P, 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), Centre national de référence des maladies pulmonaires rares [Lyon] (CRMPM), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], Centre Constitutif de Référence des Maladies Pulmonaires Rares [AP-HP Tenon], Centre national de référence des maladies pulmonaires rares [Lyon] (CRMPM)-Service de Pneumologie = Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), 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 Bretonneau, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Hôpital Avicenne [AP-HP], Institut Coeur Poumon [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Pessac, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS), Maladies génétiques d'expression pédiatrique [CHU Trousseau] (Inserm U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Nouvel Hôpital Civil de Strasbourg, Centre Hospitalier Universitaire de Reims (CHU Reims), Hôpital Pasteur [Nice] (CHU), Institut de Recherche sur le Cancer et le Vieillissement (IRCAN), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Marseille, Laboratoire d'Informatique, de Traitement de l'Information et des Systèmes (LITIS), Université Le Havre Normandie (ULH), Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA), Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Centre Hospitalier Régional d'Orléans (CHRO), Hospices Civils de Lyon (HCL), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux), Groupe Hospitalier Paris Saint-Joseph (hpsj), Services de Pneumologie, Exploration Fonctionnelle Respiratoire et Cardiologie (Hôpital Louis Pradel), Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Biopsie, Biopsy, [SDV]Life Sciences [q-bio], Fibrose pulmonaire, Pneumopathie interstitielle commune, Interstitial lung disease, Common interstitial lung disease, Pneumopathie interstitielle diffuse, Pulmonary fibrosis
Abstract

National audience; BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients’ association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

Published
2022
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41. Lung Transplantation in HIV Patients

Author

Rouzaud, C., Berastegui, C., Picard, C., Vos, R., Savale, L., Demant, X., Bertani, A., Verschuuren, E., Jaksch, P., Reed, A., Morlacchi, L., Reynaud-Gaubert, M., Prof. Jens Gottlieb, Le Pavec, J., and Groningen Institute for Organ Transplantation (GIOT)

Subjects
Pulmonary and Respiratory Medicine, Transplantation, Surgery, Cardiology and Cardiovascular Medicine
Published
2022

42. Altered pIgR/IgA mucosal immunity in bronchiolitis obliterans syndrome

Author

UCL - (MGD) Service de pneumologie, Carlier, F, Pretolani, M, Detry, B, Heddebaut, N, Planté-Bordeneuve, T, Longchampt, E, Falque, L, Reynaud-Gaubert, M, Hirschi, S, Demant, X, Mornex, J, Tissot, A, Le Pavec, J, Messika, J, Foureau, A, Vallée, A, Pilette, C, Brugière, O, The Colt Consortium, UCL - (MGD) Service de pneumologie, Carlier, F, Pretolani, M, Detry, B, Heddebaut, N, Planté-Bordeneuve, T, Longchampt, E, Falque, L, Reynaud-Gaubert, M, Hirschi, S, Demant, X, Mornex, J, Tissot, A, Le Pavec, J, Messika, J, Foureau, A, Vallée, A, Pilette, C, Brugière, O, and The Colt Consortium

Abstract

Aims: Long-term survival after lung transplantation (LT) is hampered by the occurrence of chronic lung allograft dysfunction (CLAD), manifesting as bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS). CLAD is triggered by several factors, e.g. recurrent infections. As immunoglobulin (Ig) A is crucial to ensure mucosal immunity and limit airway microbial load, we explored whether IgA and its epithelial receptor, the polymeric Ig receptor (pIgR) are impaired in BOS. Methods: Bronchoalveolar lavages (BAL, n=120) from LT recipients included in the Cohort for Lung Transplantation were collected at pre-defined timepoints prior to the diagnosis of functional stability (BOS-free, n=30) or BOS (pre-BOS, n=30), and assessed for secretory (S)-IgA. Bronchiolar epithelium pIgR expression was quantified in transbronchial biopsies from BOS-free (n=20), pre-BOS (n=19) and BOS LT recipients (n=12), as well as in end-stage BOS explants (n=15). Results: S-IgA levels were reduced in BAL from pre-BOS LT recipients versus BOS-free (16.1 vs 33.4 µg/ml, p<0.01). pIgR bronchiolar expression was reduced in transbronchial biopsies from BOS (p<0.05 vs BOS-free and pre-BOS), with further decrease in end-stage BOS explants (p<0.0001 vs BOS-free and pre-BOS). Conclusions: BAL S-IgA and pIgR decreased levels suggest that the pIgR/IgA system is impaired in BOS. This could play a pathogenic role by increasing susceptibility to local infections.

Published
2022

44. SARS‐CoV‐2 Vaccine Response in Lung Transplant Recipients: A French Multicenter Study

Author

Dauriat, G., primary, Beaumont, L., additional, Renaud-Picard, B., additional, Salpin, M., additional, Coiffard, B., additional, Danner-Boucher, I., additional, Leborgne, A., additional, Feuillet, S., additional, Penhouet, M., additional, Reynaud-Gaubert, M., additional, Gallais, F., additional, Messika, J., additional, Roux, A., additional, and Pavec, J. Le, additional

Published
2022
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46. French practical guidelines for the diagnosis and management of IPF-2021 update, short version

Author

Cottin, V, Bonniaud, P., Cadranel, J., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunes, H., Wemeau-Stervinou, L., Bergot, E., Blanchard, E., Borie, R., Bourdin, A., Chenivesse, C., Clement, A., Gomez, E., Gondouin, A., Hirschi, S., Lebargy, F., Marquette, C-H, Montani, D., Prevot, G., Quetant, S., Reynaud-Gaubert, M., Salaun, M., Sanchez, O., Trumbic, B., Berkani, K., Brillet, P-Y, Campana, M., Chalabreysse, L., Chatte, G., Debieuvre, D., Ferretti, G., Fourrier, J-M, Just, N., Kambouchner, M., Legrand, B., Le Guillou, F., Lhuillier, J-P, Mehdaoui, A., Naccache, J-M, Paganon, C., Remy-Jardin, M., Si-Mohamed, S., Terrioux, P., 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), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), RespiFIL, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Groupe de recherche clinique Biomarqueurs Théranostiques des Cancers Bronchiques Non à Petites Cellules (GRC 4 - Theranoscan), Sorbonne Université (SU), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), 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 ), CHU Pontchaillou [Rennes], Hôpital Bretonneau, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Hôpital Avicenne [AP-HP], Université Sorbonne Paris Nord, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut Coeur Poumon [CHU Lille], Service de pneumologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS), Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Trousseau [APHP], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Nouvel Hôpital Civil de Strasbourg, Hôpital Maison Blanche, Centre Hospitalier Universitaire de Reims (CHU Reims), Institut de Recherche sur le Cancer et le Vieillissement (IRCAN), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), FHU OncoAge - Pathologies liées à l’âge [CHU Nice] (OncoAge), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut de Pharmacologie Moléculaire et Cellulaire [UNIV Côte d'Azur] (UPMC)-Université Côte d'Azur (UCA), Hôpital Pasteur [Nice] (CHU), Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Hôpital Bicêtre, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire [Grenoble] (CHU), 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], Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Service de pneumologie, oncologie thoracique et soins intensifs respiratoires [Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire d'Informatique, du Traitement de l'Information et des Systèmes (LITIS), Université Le Havre Normandie (ULH), Normandie Université (NU)-Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, CHU Orléans, Groupement Hospitalier Lyon-Est (GHE), Groupe hospitalier de la région de Mulhouse Sud-Alsace (GHRMSA), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Université Grenoble Alpes (UGA), Centre Hospitalier Victor Provo, CHU Lille, Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre d'Etudes et de Recherche en Informatique Médicale [Lille] (CERIM), Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux), Centre hospitalier Saint-Joseph [Paris], Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Biopsy, Interstitial lung disease, Common interstitial lung disease, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Pulmonary fibrosis
Abstract

National audience; Background. - Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. Methods. - Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. Results. - After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. Conclusion. - These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis. (C) 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.

Published
2022
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47. French clinical practice guidelines for the diagnosis and management of lung disease with alpha 1-antitrypsin deficiency

Author

Mornex, J.-F., Balduyck, M., Bouchecareilh, M., Cuvelier, A., Epaud, R., Kerjouan, M., Le Rouzic, O., Pison, C., Plantier, L., Pujazon, M.-C., Reynaud-Gaubert, M., Toutain, A., Trumbic, B., Willemin, M.-C., Zysman, M., Brun, O., Campana, M., Chabot, F., Chamouard, V., Dechomet, M., Fauve, J., Girerd, B., Gnakamene, C., Lefrançois, S., Lombard, J.-N., Maitre, B., Maynié-François, C., Moerman, A., Payancé, A., Reix, P., Revel, D., Revel, M.-P., Schuers, M., Terrioux, P., Theron, D., Willersinn, F., Cottin, V., Mal, H., 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), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Maladies RAres du DEveloppement embryonnaire et du MEtabolisme : du Phénotype au Génotype et à la Fonction - ULR 7364 (RADEME), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Lille, Université de Lille, Bordeaux Research In Translational Oncology [Bordeaux] (BaRITOn), Université de Bordeaux (UB)-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), Groupe de Recherche sur le Handicap Ventilatoire et Neurologique (GRHVN), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institute for Research and Innovation in Biomedicine (IRIB), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de pneumologie, oncologie thoracique et soins intensifs respiratoires [Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre Hospitalier Intercommunal de Créteil (CHIC), Hôpital Pontchaillou, Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS), Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université Grenoble Alpes (UGA), Service de pneumologie [Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital Nord [CHU - APHM], Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Aix Marseille Université (AMU), Imagerie et cerveau (iBrain - Inserm U1253 - UNIV Tours ), Service de génétique [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de pneumologie et d'allergologie respiratoire [Perpignan], Centre Hospitalier Régional d'Orléans (CHRO), Défaillance Cardiovasculaire Aiguë et Chronique (DCAC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Pneumologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Centre de Référence de l’Hypertension Pulmonaire Sévère [CHU Le Kremlin Bicêtre], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Faculté de Médecine Paris-Saclay, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre)-Université Paris-Saclay, Centre Hospitalier Montélimar, CH Montélimar, Physiopathologie des Maladies du Système Nerveux Central, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon, Hôpital Jeanne de Flandre [Lille], Centre de référence des Maladies Vasculaires du Foie [Paris] (FILFOIE), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service d’Hépatologie [Hôpital Beaujon], Hôpital Beaujon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Descartes - Paris 5 (UPD5), Laboratoire d'Informatique Médicale et Ingénierie des Connaissances en e-Santé (LIMICS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Sorbonne Paris Nord, 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), and AP-HP - Hôpital Bichat - Claude Bernard [Paris]

Subjects
Déficit en alpha 1-antitrypsine, Cirrhose du foie, Emphysème pulmonaire, Bronchopneumopathie chronique obstructive, [SDV.MHEP.HEG]Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract

International audience; Contexte: Le déficit en alpha 1-antitrypsine (DAAT) est une maladie génétique autosomique récessive associée à l’état homozygote du variant Z du gène SERPINA1. Les manifestations cliniques sont un déficit sévère en alpha 1-antitrypsine, un emphysème pulmonaire et une fibrose hépatique.Méthodes: Des recommandations de prise en charge du DAAT ont été élaborées à l’initiative du centre coordonnateur de référence des maladies pulmonaires rares, sous l’égide de la Société de pneumologie de langue française, par des groupes de coordination, rédaction, et lecture, impliquant des pneumologues de divers modes d’exercice, biologistes, hépatologue, pharmacien hospitalier, conseiller en génétique, radiologues, médecins généralistes, cadre de santé, et associations de patients. La méthode d’élaboration des « Recommandations pour la pratique clinique » de la Haute autorité de santé a été suivie, incluant un vote en ligne selonune échelle Likert.Résultats: Après une analyse bibliographique, 20 recommandations ont été proposées par les groupes de travail, portant sur tous les aspects de la maladie : diagnostic biologique, bilan initial, conseils d’hygiène de vie, information, indications et modalités du traitement substitutif, dépistage.Conclusion: Ces recommandations fondées sur les preuves sont destinées à guider le diagnostic et la prise en charge pratique du DAAT.

Published
2022
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49. Association rituximab et mycophenolate mofetil chez les patients avec une pneumopathie interstitielle non spécifique : essai contrôlé randomisé en double aveugle, contre mycophenolate mofetil et placebo (Ever-Ild)

Author

Mankikian, J., primary, Caille, A., additional, Reynaud-Gaubert, M., additional, Leger, J., additional, Bonniaud, P., additional, Brillet, P.Y., additional, Cadranel, J., additional, Court-Fortune, I., additional, Crestani, B., additional, Debray, M.P., additional, Gomez, E., additional, Gondouin, A., additional, Hirschi-Santelmo, S., additional, Israel-Biet, D., additional, Jouneau, S., additional, Marquette, C.H., additional, Naccache, J.M., additional, Nunes, H., additional, Plantier, L., additional, Prevot, G., additional, Quetant, S., additional, Traclet, J., additional, Wemeau, L., additional, Angoulvant, T., additional, Cottin, V., additional, and Marchand-Adam, S., additional

Published
2022
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50. Évolution après transplantation pulmonaire des patients adultes atteints d’une pneumopathie interstitielle diffuse dans un contexte de mutation d’un gène codant pour une protéine du surfactant

Author

Bermudez, J., primary, Nathan, N., additional, Coiffard, B., additional, Roux, A., additional, Hirschi, S., additional, Degot, T., additional, Bunel, V., additional, Le Pavec, J., additional, Macey, J., additional, Le Borgne, A., additional, Legendre, M., additional, Cottin, V., additional, Thomas, P., additional, Borie, R., additional, and Reynaud-Gaubert, M., additional

Published
2022
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