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2. 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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3. 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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4. 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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8. Efficacité du plasma de patients convalescents chez les patients transplantés pulmonaires infectés par le SARS-CoV2 : étude multicentrique française

Author

Chaudhry, A., primary, Gallais, F., additional, Hirschi, S., additional, Degot, T., additional, Verdiere, S. Colin De, additional, Villeneuve, T., additional, Langlard, D. Horeau, additional, Chatron, E., additional, Blanchard, E., additional, Kessler, R., additional, and Renaud-Picard, B., additional

Published
2024
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10. Airway microbiota signals anabolic and catabolic remodeling in the transplanted lung

Author

Jougon, J., Velly, J.-F., Rozé, H., Blanchard, E., Dromer, C., Antoine, M., Cappello, M., Ruiz, M., Sokolow, Y., Vanden Eynden, F., Van Nooten, G., Barvais, L., Berré, J., Brimioulle, S., De Backer, D., Créteur, J., Engelman, E., Huybrechts, I., Ickx, B., Preiser, T.J.C., Tuna, T., Van Obberghe, L., Vancutsem, N., Vincent, J.-L., De Vuyst, P., Etienne, I., Féry, F., Jacobs, F., Knoop, C., Vachiéry, J.L., Van den Borne, P., Wellemans, I., Amand, G., Collignon, L., Giroux, M., Angelescu, D., Chavanon, O., Hacini, R., Pirvu, A., Porcu, P., Albaladejo, P., Allègre, C., Bataillard, A., Bedague, D., Briot, E., Casez-Brasseur, M., Colas, D., Dessertaine, G., Durand, M., Francony, G., Hebrard, A., Marino, M.R., Oummahan, B., Protar, D., Rehm, D., Robin, S., Rossi-Blancher, M., Augier, C., Bedouch, P., Boignard, A., Bouvaist, H., Briault, A., Camara, B., Claustre, J., Chanoine, S., Dubuc, M., Quétant, S., Maurizi, J., Pavèse, P., Pison, C., Saint-Raymond, C., Wion, N., Chérion, C., Grima, R., Jegaden, O., Maury, J.-M., Tronc, F., Flamens, C., Paulus, S., Mornex, J.-F., Philit, F., Senechal, A., Glérant, J.-C., Turquier, S., Gamondes, D., Chalabresse, L., Thivolet-Bejui, F., Barnel, C., Dubois, C., Tiberghien, A., Le Pimpec-Barthes, F., Bel, A., Mordant, P., Achouh, P., Boussaud, V., Guillemain, R., Méléard, D., Bricourt, M.O., Cholley, B., Pezella, V., Brioude, G., D'Journo, X.B., Doddoli, C., Thomas, P., Trousse, D., Dizier, S., Leone, M., Papazian, L., Bregeon, F., Basire, A., Coltey, B., Dufeu, N., Dutau, H., Garcia, S., Gaubert, J.Y., Gomez, C., Laroumagne, S., Nieves, A., Picard, L.C., Reynaud-Gaubert, M., Secq, V., Mouton, G., Baron, O., Lacoste, P., Perigaud, C., Roussel, J.C., Danner, I., Haloun, A., Magnan, A., Tissot, A., Lepoivre, T., Treilhaud, M., Botturi-Cavaillès, K., Brouard, S., Danger, R., Loy, J., Morisset, M., Pain, M., Pares, S., Reboulleau, D., Royer, P.-J., Fabre, D., Fadel, E., Mercier, O., Mussot, S., Stephan, F., Viard, P., Cerrina, J., Dorfmuller, P., Ghigna, S.M., Hervén, Ph., Le Roy Ladurie, F., Le Pavec, J., Thomas de Montpreville, V., Lamrani, L., Castier, Y., Cerceau, P., Augustin, P., Jean-Baptiste, S., Boudinet, S., Montravers, P., Brugière, O., Dauriat, G., Jébrak, G., Mal, H., Marceau, A., Métivier, A.-C., Thabut, G., Lhuillier, E., Dupin, C., Bunel, V., Falcoz, P., Massard, G., Santelmo, N., Ajob, G., Collange, O., Helms, O., Hentz, J., Roche, A., Bakouboula, B., Degot, T., Dory, A., Hirschi, S., Ohlmann-Caillard, S., Kessler, L., Kessler, R., Schuller, A., Bennedif, K., Vargas, S., Stauder, J., Ali-Azouaou, S., Bonnette, P., Chapelier, A., Puyo, P., Sage, E., Bresson, J., Caille, V., Cerf, C., Devaquet, J., Dumans-Nizard, V., Felten, M.-L., Fischler, M., Si Larbi, A.-G., Leguen, M., Ley, L., Liu, N., Trebbia, G., De Miranda, S., Douvry, B., Gonin, F., Grenet, D., Hamid, A.M., Neveu, H., Parquin, F., Picard, C., Roux, A., Stern, M., Bouillioud, F., Cahen, P., Colombat, M., Dautricourt, C., Delahousse, M., D'Urso, B., Gravisse, J., Guth, A., Hillaire, S., Honderlick, P., Lequintrec, M., Longchampt, E., Mellot, F., Scherrer, A., Temagoult, L., Tricot, L., Vasse, M., Veyrie, C., Zemoura, L., Berjaud, J., Brouchet, L., Dahan, M., Mathe, F.O., Benahoua, H., DaCosta, M., Serres, I., Merlet-Dupuy, V., Grigoli, M., Didier, A., Murris, M., Crognier, L., Fourcade, O., Krueger, T., Ris, H.B., Gonzalez, M., Jolliet, Ph., Marcucci, C., Chollet, M., Gronchi, F., Courbon, C., Berutto, C., Manuel, O., Koutsokera, A., Aubert, J.-D., Nicod, L.P., Mouraux, S., Bernasconi, E., Pattaroni, C., Marsland, B.J., Soccal, P.M., Rochat, T., Lücker, L.M., Hillinger, S., Inci, I., Weder, W., Schuepbach, R., Zalunardo, M., Benden, C., Schuurmans, M.M., Gaspert, A., Holzmann, D., Müller, N., Schmid, C., Vrugt, B., Fritz, A., Maier, D., Deplanche, K., Koubi, D., Ernst, F., Paprotka, T., Schmitt, M., Wahl, B., Boissel, J.-P., Olivera-Botello, G., Trocmé, C., Toussaint, B., Bourgoin-Voillard, S., Sève, M., Benmerad, M., Siroux, V., Slama, R., Auffray, C., Charron, D., Lefaudeux, D., Pellet, J., Mouraux, Stéphane, Bernasconi, Eric, Pattaroni, Céline, Koutsokera, Angela, Aubert, John-David, Claustre, Johanna, Pison, Christophe, Royer, Pierre-Joseph, Magnan, Antoine, Kessler, Romain, Benden, Christian, Soccal, Paola M., Marsland, Benjamin J., and Nicod, Laurent P.

Published
2018
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11. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

13. (293) Altered Pigr/Iga Mucosal Immunity in Bronchiolitis Obliterans Syndrome

Author

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

Published
2023
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14. (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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15. Phosphorylated S6 Ribosomal Protein as an Additional Marker of Antibody-Mediated Rejection in Lung Allografts: A Multicentre Study

Author

Lunardi, F., primary, Vedovelli, L., additional, Pezzuto, F., additional, Pavec, J. Le, additional, Dorfmuller, P., additional, Ivanovic, M., additional, Pena, T., additional, Wassilew, K., additional, Perch, M., additional, Hirschi, S., additional, Chenard, M., additional, Neil, D., additional, Montero-Fernandez, M., additional, Rice, A., additional, Cozzi, E., additional, Rea, F., additional, Levine, D.J., additional, Roux, A., additional, Goddard, M., additional, Fishbein, G.A., additional, and Calabrese, F., additional

Published
2023
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17. 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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18. 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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19. Prévalence du cancer du poumon dans une cohorte de patients porteurs d’une mutation d’un gène lié au surfactant

Author

Brudon, A., primary, Legendre, M., additional, Mageau, A., additional, Nathan, N., additional, Bermudez, J., additional, Bouvry, D., additional, Cadranel, J., additional, Cazes, A., additional, Crestani, B., additional, Dégot, T., additional, Delestrain, C., additional, Diesler, R., additional, Epaud, R., additional, Debray, M.P., additional, Fanen, P., additional, Gaubert, A., additional, Manali, E., additional, Gondouin, A., additional, Guillaumot, A., additional, Hirschi, S., additional, Leroy, S., additional, Marchand-Adam, S., additional, Nunes, H., additional, Amselem, S., additional, Picard, C., additional, Prevot, G., additional, Reynaud, M., additional, De Vuyst, P., additional, Wémeau-Stervinou, L., additional, Zalcman, G., additional, Cottin, V., additional, and Borie, R., additional

Published
2023
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22. Coordination régionale et suivi prospectif du traitement prophylactique et curatif de 301 patients traités par anticorps monoclonaux anti-SARS-CoV-2

Author

Licinio, P., primary, Weil, M., additional, Morel, M.H., additional, Braun, D. Marin, additional, Gourieux, B., additional, Klein, C., additional, Sordet, C., additional, Chatelus, E., additional, Felten, R., additional, Meyer, A., additional, Sibilia, J., additional, Hirschi, S., additional, Degot, T., additional, Picard, B. Renaud, additional, Virot, E., additional, Michard, B., additional, Kremer, L.D., additional, Bigault, K., additional, Cevallos, R., additional, Lefebvre, N., additional, Hansmann, Y., additional, Bronner, C., additional, Rybarczyk-Vigouret, M.C., additional, Pain, L., additional, and Gottenberg, J.E., additional

Published
2022
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23. 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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24. [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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25. High Prevalence and impact of lung cancer in Surfactant related gene mutation carriers

Author

BRUDON, A, primary, Nathan, N, additional, Legendre, M, additional, Amselem, S, additional, Bermudez, J, additional, Bouvry, D, additional, Cadranel, J, additional, Cazes, A, additional, Degot, T, additional, Delestrain, C, additional, Diesler, R, additional, Epaud, R, additional, Fanen, P, additional, Gondouin, A, additional, Guillaumot, A, additional, Hirschi, S, additional, Leroy, S, additional, Marchand-Adam, S, additional, Nunes, H, additional, Picard, C, additional, Prévot, G, additional, Reynaud, M, additional, De Vuyst, P, additional, Wemeau, L, additional, Crestani, B, additional, Zalcman, G, additional, Cottin, V, additional, and Borie, R, additional

Published
2022
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26. 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

27. 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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30. Bronchiolite chronique idiopathique : cohorte française rétrospective multicentrique

Author

Nasser, M., primary, Uzunhan, Y., additional, Marchand Adam, S., additional, Hirschi, S., additional, Israël-Biet, D., additional, Jouneau, S., additional, Lebargy, F., additional, Justet, A., additional, Taillé, C., additional, Wemeau, L., additional, Valentin, V., additional, Borie, R., additional, Godbert, B., additional, Gagnadoux, F., additional, Guilleminault, L., additional, Nunes, H., additional, and Cottin, V., additional

Published
2022
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31. 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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35. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation

Author

Justet, A. Klay, D. Porcher, R. Cottin, V. Ahmad, K. Molina, M.M. Nunes, H. Reynaud-Gaubert, M. Naccache, J.M. Manali, E. Froidure, A. Jouneau, S. Wemeau, L. Andrejak, C. Gondouin, A. Hirschi, S. Blanchard, E. Bondue1, B. Bonniaud, P. Tromeur, C. Prévot, G. Marchand-Adam, S. Funke-Chambour, M. Gamez, A.S. Ba, I. Papiris, S. Grutters, J. Crestani, B. Van Moorsel, C. Kannengiesser, C.

Published
2021

37. Association between influenza vaccination and the occurrence of acute exacerbation inidiopathic pulmonary fibrosis

Author

Sese, L., primary, Caliez, J., additional, Cottin, V., additional, Israel-Biet, D., additional, Crestani, B., additional, Guillot-Dudoret, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maître, B., additional, Prévot, G., additional, Marchan-Adam, S., additional, Hirschi, S., additional, Dury, S., additional, Giraud, V., additional, Gondouin, A., additional, Bonniaud, P., additional, Traclet, J., additional, Juvin, K., additional, Borie, R., additional, Gille, T., additional, Carton, Z., additional, Valeyre, D., additional, and Nunes, H., additional

Published
2021
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38. Low income and progression free survival in idiopathic pulmonary fibrosis: An association to uncover

Author

Sese, L., primary, Caliez, J., additional, Annesi-Maesano, I., additional, Cottin, V., additional, Pesce, G., additional, Didier, M., additional, Carton, Z., additional, Israel-Biet, D., additional, Crestani, B., additional, Guillot Dudoret, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maître, B., additional, Prévot, G., additional, Marchand-Adam, S., additional, Hirschi, S., additional, Dury, S., additional, Giraud, V., additional, Gondouin, A., additional, Bonniaud, P., additional, Traclet, J., additional, Juvin, K., additional, Borie, R., additional, Bernaudin, J.F., additional, Valeyre, D., additional, Cavalin, C., additional, and Nunes, H., additional

Published
2021
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39. Gender differences in idiopathic pulmonary fibrosis: Are men and women equal or not?

Author

Sese, L., primary, Nunes, H., additional, Cottin, V., additional, Israel-Biet, D., additional, Crestani, B., additional, Guillot Dudoret, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maître, B., additional, Prévot, G., additional, Marchand-Adam, S., additional, Hirschi, S., additional, Dury, S., additional, Giraud, V., additional, Gondouin, A., additional, Bonniaud, P., additional, Traclet, J., additional, Juvin, K., additional, Borie, R., additional, Carton, Z., additional, Caliez, J., additional, Freynet, O., additional, Gille, T., additional, Planes, C., additional, Valeyre, D., additional, and Uzunhan, Y., additional

Published
2021
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40. Demographics and Baseline Characteristics of Patients with Idiopathic Pulmonary Fibrosis (IPF) in a Real-World Setting: Results of 847 Patients Enrolled in the Radico-ILD Cohort in France

Author

Cottin, V, Jouneau, S, Crestani, B, Nunes, H, Wemeau, L, Bonniaud, P, Israël-Biet, D, Reynaud-Gaubert, M, Dalphin, J, Naccache, J, Marchand-Adam, S, Quetant, S, Montani, D, Hirschi, S, Chevereau, M, Dufaure-Garé, I, Clement, A, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), CHU Pontchaillou [Rennes], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Avicenne [AP-HP], Université de Lille, 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), Aix Marseille Université (AMU), Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Tours (UT), Université Grenoble Alpes (UGA), Hôpital Bicêtre, Université de Strasbourg (UNISTRA), Rare Disease Cohorts (RaDiCo), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], 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 Trousseau [APHP], and ANR-10-COHO-0003,RADICO,Cohorte nationale maladies rares(2010)

Subjects
[SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract

International audience; Rationale: Idiopathic pulmonary fibrosis (IPF) is a rare condition and few epidemiological dataare available in France. This specific research project aims to describe characteristics of treatedIPF patients and the impact of antifibrotic treatments in terms of morbidity and mortality in theFrench real-life setting. Methods: The French RaDiCo (Rare Disease Cohort)-ILD (idiopathicInterstitial Lung Diseases) registry is an ongoing observational study initiated in June 2017, witha sub-analysis of IPF patients. This longitudinal long-term cohort includes pediatric and adultpatients with ILD and is supported by the national network of reference and competence centersfor rare pulmonary diseases. IPF was diagnosed using international ATS/ERS 2011 criteria witha diagnosis of IPF or working diagnosis of IPF by multidisciplinary discussion. Here, we presentthe baseline data of IPF patients. Results: Between June 15th 2017 and September 4th 2019,1246 ILD patients were enrolled in the RaDiCo-ILD registry from 18 centers, including 847 withIPF (68%). IPF patients were mostly male (82.7%), with a mean age of 72.5 ± 9 years at inclusionand a mean BMI of 26.8 ± 4.3; 44.6 % of IPF patients included were incident cases, with amedian length between diagnosis and inclusion of 8.9 months (Q1=0.9 and Q3=26.4); 25.3% hada biopsy. The mean FVC at IPF diagnosis was 73.4 ± 25.0 % of predicted value (n=561), and themean DLCO at IPF diagnosis was 39.6 ± 18.2 % predicted value (n=498). Among patients withavailable information on anti-fibrotic treatment, 347 had been treated (at least one dose) withnintedanib, and 312 with pirfenidone; among patients treated with antifibrotics, 113 were treatedwith both treatments sequentially. Conclusions: The RaDiCo-ILD registry provides accurate real-world data on the demographics of patients with IPF in France. It will generate a long-term follow-up and will be an invaluable tool to describe the natural history and progression of patients withIPF in real-life conditions.

Published
2020

41. Infinitix-BOS Trial: Multi-Center, Randomised, Double-Blind Placebo-Controlled Trial of Nintedanib in Lung Transplant Recipients with Bronchiolitis Obliterans Syndrome (BOS) Grade 0-p and Grade 1-2

Author

Brugiere, O., primary, Picard, C., additional, Messika, J., additional, Weisenburger, G., additional, Bunel, V., additional, Demant, X., additional, Bon, c., additional, Macey, C., additional, Le Pavec, J., additional, Dauriat, G., additional, Crutu, A., additional, Hirschi, S., additional, Renaud Picard, b., additional, Degot, T., additional, Reynaud-Gaubert, M., additional, Coiffard, B., additional, Coltey, B., additional, Pison, C., additional, Raymond, C. Saint, additional, Briault, a., additional, Hamid, A., additional, Beaumont, L., additional, and Roux, A., additional

Published
2021
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42. Airway Complications in Lung Transplant Recipients with Telomere-Related Interstitial Lung Disease

Author

Choi, B., primary, Messika, J., additional, Courtwright, A., additional, Mornex, J., additional, Hirschi, S., additional, Roux, A., additional, Le Pavec, J., additional, Quêtant, S., additional, Froidure, A., additional, Lazor, R., additional, Reynaud-Gaubert, M., additional, Le Borgne, A., additional, Goldberg, H., additional, El-Chemaly, S., additional, and Borie, R., additional

Published
2021
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45. Différences entre les genres dans la fibrose pulmonaire idiopathique : les hommes et les femmes sont-ils égaux ?

Author

Sese, L., primary, Nunes, H., additional, Cottin, V., additional, Israel-Biet, D., additional, Crestani, B., additional, Guillot Dudoret, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maitre, B., additional, Prevot, G., additional, Marchand-Adam, S., additional, Hirschi, S., additional, Dury, S., additional, Giraud, V., additional, Gondouin, A., additional, Bonniaud, P., additional, Traclet, J., additional, Juvin, K., additional, Borie, R., additional, Carton, Z., additional, Caliez, J., additional, Freynet, O., additional, Gille, T., additional, Planes, C., additional, Valeyre, D., additional, and Uzunhan, Y., additional

Published
2021
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46. Antifibrosants: risque post-opératoire chez les patients transplantés pour une fibrose pulmonaire idiopathique

Author

Moncomble, E., primary, Weisenburger, G., additional, Picard, C., additional, Degot, T., additional, Reynaud-Gaubert, M., additional, Nieves, A., additional, Mornex, J.F., additional, Dauriat, G., additional, Messika, J., additional, Godet, C., additional, Hirschi, S., additional, Le Pavec, J., additional, Borie, R., additional, Mordant, P., additional, Mal, H., additional, and Bunel, V., additional

Published
2021
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47. Rôle de la vaccination antigrippale sur le risque d’exacerbation aiguë dans la fibrose pulmonaire idiopathique

Author

Sese, L., primary, Alari, A., additional, Pesce, G., additional, Caliez, J., additional, Cottin, V., additional, Israel-Biet, D., additional, Crestani, B., additional, Guillot, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maitre, B., additional, Prevot, G., additional, Marchand-Adam, S., additional, Hirschi, S., additional, Dury, S., additional, Giraud, V., additional, Gondouin, A., additional, Bonniaud, P., additional, Traclet, J., additional, Juvin, K., additional, Borie, R., additional, Gille, T., additional, Carton, Z., additional, Valeyre, D., additional, and Nunes, H., additional

Published
2021
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49. Essai multicentrique, randomisé double aveugle contre placebo, évaluant l’efficacité du Nintedanib dans le traitement du syndrome de bronchiolite oblitérante grade 0-p et grade 1-2 chez les patients transplantés pulmonaires

Author

Brugière, O., primary, Couffignal, C., additional, Maurer, Y., additional, Messika, J., additional, Weisenburger, G., additional, Bunel, V., additional, Demant, X., additional, Bon, C., additional, Macey, C., additional, Le Pavec, J., additional, Dauriat, G., additional, Crutu, A., additional, Hirschi, S., additional, Renaud-Picard, B., additional, Degot, T., additional, Reynaud-Gaubert, M., additional, Coltey, B., additional, Pison, C., additional, Saint Raymond, C., additional, Briot, A., additional, Makhlouf, S., additional, Beaumont, L., additional, Picard, C., additional, and Antoine, R., additional

Published
2021
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