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4. Long-term effect of nintedanib treatment in 11 countries of Europe and Asia

Author

Koziar Vasakova, M, primary, Sterclova, M, additional, Mogulkoc Bishop, N, additional, Lewandowska, K, additional, Müller, V, additional, Kramer, M, additional, Hajkova, M, additional, Jovanovic, D, additional, Studnicka, M, additional, Tekavec-Trkanjec, J, additional, Penev, A, additional, Ovesna, P, additional, and Gregor, J, additional

Published
2022
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6. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study

Author

Walsh S. L. F., Maher T. M., Kolb M., Poletti V., Nusser R., Richeldi L., Vancheri C., Wilsher M. L., Antoniou K. M., Behr J., Bendstrup E., Brown K., Calandriello L., Corte T. J., Cottin V., Crestani B., Flaherty K., Glaspole I., Grutters J., Inoue Y., Kokosi M., Kondoh Y., Kouranos V., Kreuter M., Johannson K., Judge E., Ley B., Margaritopoulos G., Martinez F. J., Molina-Molina M., Morais A., Nunes H., Raghu G., Ryerson C. J., Selman M., Spagnolo P., Taniguchi H., Tomassetti S., Valeyre D., Wijsenbeek M., Wuyts W., Hansell D., Wells A., Zhu P. S., Yuan Y., Yoshito Fukuda C., Yoshimatsu Y., Xaubet A., Wong A. M., White P., Westney G., West A., Wessendorf T., Waseda Y., Wang C., Vienna J. M., Videnovic Ivanov J., Vicens Zygmunt V., Venero Caceres M. C., Velasquez Pinto G., Veitch E., Vasakova M., Varone F., Varela B. E., Van Hal P., Van De Ven M., Van Der Lee I., Van Den Toorn L., Urrutia Gajate A., Urban J., Ugarte Fornell L. G., Tzouvelekis A., Twohig K., Turner A., Trujillo S., Triani A., Traila D., Torres V., Tomioka H., Tomii K., Tomic R., Toma C., Tokgoz Akyil F., Tobino K., Tobar R., Tiwari A., Tibana R., Tian X., Thillai M., Tham W., Teo F., Tekavec Trkanjec J., Teixeira P., Tarpey D., Tapias L., Tanizawa K., Tanino Y., Takada T., Tabaj G., Szolnoki E., Swarnakar R., Strambu I., Sterclova M., Spinks K., Soo C. I., Soltani A., Solanki S., Sobh E., Soares M. R., Smith J., Smith B., Slocum P., Slabbynck H., Sivokozov I., Shifren A., Shen S. M., Sharp C., Shanmuganathan A., Sebastiani A., Scarlata S., Savas R., Sasaki S., Santeliz J., Santana ANC., Sanchez R., Salinas M., Saito S., Ryan F., Royo Prats J. A., Rosi E., Rokadia H., Robles Perez A., Rivera Ortega P., Rio Ramirez M., Righetti S., Reichner C., Ravaglia C., Ratanawatkul P., Ramalingam V., Rajasekaran A., Radzikowska E., Ra S. W., Quadrelli S., Precerutti J., Prasad J., Popa D., Pizzalato S., Piotrowski W., Pineiro A., Piloni D., Peros Golubicic T., Perez R., Pereira C., Pereira B., Perch M., Patel N., Patel D., Papanikolaou I., Papakosta D., Panselinas E., Pang Y. K., Pandya P., Padrao E., Ozdemir Kumbasar O., Overbeek M. J., Otto Minasian A., O'Riordan D., Ora J., Oldham J., Okutan O., Ohshimo S., Oguzulgen I. K., Ogura T., O'Donnell T., O'Dochartaigh C., O'Beirne S., Novikova L., Novelli L., Noth I., Nogueira Mendes Neto N., Niroumand M., Nieto A., Neves A., Nambiar A., Nair S., Nadama R., Murtagh E., Mura M., Muller Quernheim J., Mukhopadhyay A., Mukherjee S., Morisset J., Moran O., Mooney J., Moller J., Mogulkoc N., Miyamoto A., Milenkovic B., Mette S., Mejia M., Mei F., Mazzei M., Matsuda T., Mason C., Martinez Frances M., Mannarino S., Mancuzo E., Malli F., Malhotra P., Maillo M., Maia J., Mahdavian M., Madsen F., Luckhardt T., Lucht W., Low S. Y., Lopez Miguel C. P., Lipchik R., Levy S., Levin K., Lee K. L., Lederer D., Lammi M. R., Kwan H. Y., Kukreja S., Kruavit A., Kotecki M., Kolilekas L., Knoop H., Kiyan E., Kishaba T., King Biggs M., Khor Y. H., Khan A., Khalil N., Kedia R., Kebba N., Kawano Dourado L., Kapitan K., Kan C. D., Kalyoncu A. F., Kalluri M., Kabasakal Y., Jyothula S., Juretschke M. A., Jovanovic D., Jonkers R., Jo H., Izumi S., Ishii H., Ikeda S., Ibrahim A., Hyldgaard C., Hunninghake G., Huie T., Hufton A., Hu X., Hseih W. C., Hoyos R., Hoyles R., Holguin Rodriguez O., Hogan M. P., Hodgson U., Hilkin Sogoloff H., Herrera E., Henry B. M., Hellemons M., Hecimovic A., Hayashi R., Hart S., Harari S., Haney S., Hambly N., Hakkim R., Gutierrez M., Gripaldo R., Gomez A., Goh N., Godoy R., Gilbert C., Giannarakis I., Gasparini S., Garcha P., Furtado S., Fois A., Flood Page P., Fletcher S., Fiss E., Figueroa Casas J., Figueroa Casas M., Fiddler C. A., Ferrara G., Fernandez Casares M., Felton C., Faverio P., Fabro A. T., Estrada A., Errhalt P., Enomoto N., Enghelmayer J. I., El Kersh K., Eiger G., Dubaniewicz A., Drakopanagiotakis F., Disayabutr S., Dijkstra A., Diaz Patino J. C., Diaz Castanon J. J., Dhooria S., Dhasmana D. J., De Rosa M., De Luca S., Delobbe A., Delgado D., Delgado C., De La Fuente I., De Kruif M., De Gier M., De Andrade J., Davidsen J. R., Daoud B., Dalhoff K., Cotera Solano J. V., Costa A. N., Coronel S., Confalonieri M., Conemans L., Comellas A., Colella S., Clemente S., Clark J., Ciuffreda M., Chung C. L., Chong S. G., Chirita D., Chen P. L., Chaudhuri N., Chambers D., Chalmers G., Chairman D., Chai G. T., Chacon Chaves R., Cetinsu V., Ceruti M., Ceballos Zuniga C. O., Castillo D., Carbone R. G., Caminati A., Callejas Gonzalez F. J., Butler M., Bustos C., Bukowczan M., Buendia I., Brunetti G., Brockway B., Bresser P., Breseghello J., Bouros D., Botero Zaccour J. A., Borzone G., Borie R., Blum H. C., Blank J., Biswas A., Bennett D., Benjamin M., Belaconi I. N., Beirne P., Beckert L., Bastiampillai S., Bascom R., Bartholmai B., Barros M., Ban AYL., Balestro E., Baldi B., Baddini Martinez J., Baburao A., Babu S., Averyanov A., Avdeev S., Athanazio R., Atahan E., Asuquo B., Assayag D., Antuni J., Antillon S., Anderson K. C., Anderson A., Alwani F., Altinisik G., Alsouofi N., Allam J. S., Al Jahdali H., Al Farttoosi A., Alfaro T., Al Busaidi N., Alavi Foumani A., Agreda Vedia M. G., Agarwal A., Afridi F., Adeyeye O. O., Adegunsoye A., Adamali H., Abedini A., Walsh, S. L. F., Maher, T. M., Kolb, M., Poletti, V., Nusser, R., Richeldi, L., Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K., Calandriello, L., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K., Glaspole, I., Grutters, J., Inoue, Y., Kokosi, M., Kondoh, Y., Kouranos, V., Kreuter, M., Johannson, K., Judge, E., Ley, B., Margaritopoulos, G., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Ryerson, C. J., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W., Hansell, D., Wells, A., Zhu, P. S., Yuan, Y., Yoshito Fukuda, C., Yoshimatsu, Y., Xaubet, A., Wong, A. M., White, P., Westney, G., West, A., Wessendorf, T., Waseda, Y., Wang, C., Vienna, J. M., Videnovic Ivanov, J., Vicens Zygmunt, V., Venero Caceres, M. C., Velasquez Pinto, G., Veitch, E., Vasakova, M., Varone, F., Varela, B. E., Van Hal, P., Van De Ven, M., Van Der Lee, I., Van Den Toorn, L., Urrutia Gajate, A., Urban, J., Ugarte Fornell, L. G., Tzouvelekis, A., Twohig, K., Turner, A., Trujillo, S., Triani, A., Traila, D., Torres, V., Tomioka, H., Tomii, K., Tomic, R., Toma, C., Tokgoz Akyil, F., Tobino, K., Tobar, R., Tiwari, A., Tibana, R., Tian, X., Thillai, M., Tham, W., Teo, F., Tekavec Trkanjec, J., Teixeira, P., Tarpey, D., Tapias, L., Tanizawa, K., Tanino, Y., Takada, T., Tabaj, G., Szolnoki, E., Swarnakar, R., Strambu, I., Sterclova, M., Spinks, K., Soo, C. I., Soltani, A., Solanki, S., Sobh, E., Soares, M. R., Smith, J., Smith, B., Slocum, P., Slabbynck, H., Sivokozov, I., Shifren, A., Shen, S. M., Sharp, C., Shanmuganathan, A., Sebastiani, A., Scarlata, S., Savas, R., Sasaki, S., Santeliz, J., Santana, Anc., Sanchez, R., Salinas, M., Saito, S., Ryan, F., Royo Prats, J. A., Rosi, E., Rokadia, H., Robles Perez, A., Rivera Ortega, P., Rio Ramirez, M., Righetti, S., Reichner, C., Ravaglia, C., Ratanawatkul, P., Ramalingam, V., Rajasekaran, A., Radzikowska, E., Ra, S. W., Quadrelli, S., Precerutti, J., Prasad, J., Popa, D., Pizzalato, S., Piotrowski, W., Pineiro, A., Piloni, D., Peros Golubicic, T., Perez, R., Pereira, C., Pereira, B., Perch, M., Patel, N., Patel, D., Papanikolaou, I., Papakosta, D., Panselinas, E., Pang, Y. K., Pandya, P., Padrao, E., Ozdemir Kumbasar, O., Overbeek, M. J., Otto Minasian, A., O'Riordan, D., Ora, J., Oldham, J., Okutan, O., Ohshimo, S., Oguzulgen, I. K., Ogura, T., O'Donnell, T., O'Dochartaigh, C., O'Beirne, S., Novikova, L., Novelli, L., Noth, I., Nogueira Mendes Neto, N., Niroumand, M., Nieto, A., Neves, A., Nambiar, A., Nair, S., Nadama, R., Murtagh, E., Mura, M., Muller Quernheim, J., Mukhopadhyay, A., Mukherjee, S., Morisset, J., Moran, O., Mooney, J., Moller, J., Mogulkoc, N., Miyamoto, A., Milenkovic, B., Mette, S., Mejia, M., Mei, F., Mazzei, M., Matsuda, T., Mason, C., Martinez Frances, M., Mannarino, S., Mancuzo, E., Malli, F., Malhotra, P., Maillo, M., Maia, J., Mahdavian, M., Madsen, F., Luckhardt, T., Lucht, W., Low, S. Y., Lopez Miguel, C. P., Lipchik, R., Levy, S., Levin, K., Lee, K. L., Lederer, D., Lammi, M. R., Kwan, H. Y., Kukreja, S., Kruavit, A., Kotecki, M., Kolilekas, L., Knoop, H., Kiyan, E., Kishaba, T., King Biggs, M., Khor, Y. H., Khan, A., Khalil, N., Kedia, R., Kebba, N., Kawano Dourado, L., Kapitan, K., Kan, C. D., Kalyoncu, A. F., Kalluri, M., Kabasakal, Y., Jyothula, S., Juretschke, M. A., Jovanovic, D., Jonkers, R., Jo, H., Izumi, S., Ishii, H., Ikeda, S., Ibrahim, A., Hyldgaard, C., Hunninghake, G., Huie, T., Hufton, A., Hu, X., Hseih, W. C., Hoyos, R., Hoyles, R., Holguin Rodriguez, O., Hogan, M. P., Hodgson, U., Hilkin Sogoloff, H., Herrera, E., Henry, B. M., Hellemons, M., Hecimovic, A., Hayashi, R., Hart, S., Harari, S., Haney, S., Hambly, N., Hakkim, R., Gutierrez, M., Gripaldo, R., Gomez, A., Goh, N., Godoy, R., Gilbert, C., Giannarakis, I., Gasparini, S., Garcha, P., Furtado, S., Fois, A., Flood Page, P., Fletcher, S., Fiss, E., Figueroa Casas, J., Figueroa Casas, M., Fiddler, C. A., Ferrara, G., Fernandez Casares, M., Felton, C., Faverio, P., Fabro, A. T., Estrada, A., Errhalt, P., Enomoto, N., Enghelmayer, J. I., El Kersh, K., Eiger, G., Dubaniewicz, A., Drakopanagiotakis, F., Disayabutr, S., Dijkstra, A., Diaz Patino, J. C., Diaz Castanon, J. J., Dhooria, S., Dhasmana, D. J., De Rosa, M., De Luca, S., Delobbe, A., Delgado, D., Delgado, C., De La Fuente, I., De Kruif, M., De Gier, M., De Andrade, J., Davidsen, J. R., Daoud, B., Dalhoff, K., Cotera Solano, J. V., Costa, A. N., Coronel, S., Confalonieri, M., Conemans, L., Comellas, A., Colella, S., Clemente, S., Clark, J., Ciuffreda, M., Chung, C. L., Chong, S. G., Chirita, D., Chen, P. L., Chaudhuri, N., Chambers, D., Chalmers, G., Chairman, D., Chai, G. T., Chacon Chaves, R., Cetinsu, V., Ceruti, M., Ceballos Zuniga, C. O., Castillo, D., Carbone, R. G., Caminati, A., Callejas Gonzalez, F. J., Butler, M., Bustos, C., Bukowczan, M., Buendia, I., Brunetti, G., Brockway, B., Bresser, P., Breseghello, J., Bouros, D., Botero Zaccour, J. A., Borzone, G., Borie, R., Blum, H. C., Blank, J., Biswas, A., Bennett, D., Benjamin, M., Belaconi, I. N., Beirne, P., Beckert, L., Bastiampillai, S., Bascom, R., Bartholmai, B., Barros, M., Ban, Ayl., Balestro, E., Baldi, B., Baddini Martinez, J., Baburao, A., Babu, S., Averyanov, A., Avdeev, S., Athanazio, R., Atahan, E., Asuquo, B., Assayag, D., Antuni, J., Antillon, S., Anderson, K. C., Anderson, A., Alwani, F., Altinisik, G., Alsouofi, N., Allam, J. S., Al Jahdali, H., Al Farttoosi, A., Alfaro, T., Al Busaidi, N., Alavi Foumani, A., Agreda Vedia, M. G., Agarwal, A., Afridi, F., Adeyeye, O. O., Adegunsoye, A., Adamali, H., Abedini, A., National Institute for Health Research, British Lung Foundation, Walsh, S, Maher, T, Kolb, M, Poletti, V, Nusser, R, Richeldi, L, Vancheri, C, Wilsher, M, Antoniou, K, Behr, J, Bendstrup, E, Brown, K, Calandriello, L, Corte, T, Cottin, V, Crestani, B, Flaherty, K, Glaspole, I, Grutters, J, Inoue, Y, Kokosi, M, Kondoh, Y, Kouranos, V, Kreuter, M, Johannson, K, Judge, E, Ley, B, Margaritopoulos, G, Martinez, F, Molina-Molina, M, Morais, A, Nunes, H, Raghu, G, Ryerson, C, Selman, M, Spagnolo, P, Taniguchi, H, Tomassetti, S, Valeyre, D, Wijsenbeek, M, Wuyts, W, Hansell, D, Wells, A, Zhu, P, Yuan, Y, Yoshito Fukuda, C, Yoshimatsu, Y, Xaubet, A, Wong, A, White, P, Westney, G, West, A, Wessendorf, T, Waseda, Y, Wang, C, Vienna, J, Videnovic Ivanov, J, Vicens Zygmunt, V, Venero Caceres, M, Velasquez Pinto, G, Veitch, E, Vasakova, M, Varone, F, Varela, B, Van Hal, P, Van De Ven, M, Van Der Lee, I, Van Den Toorn, L, Urrutia Gajate, A, Urban, J, Ugarte Fornell, L, Tzouvelekis, A, Twohig, K, Turner, A, Trujillo, S, Triani, A, Traila, D, Torres, V, Tomioka, H, Tomii, K, Tomic, R, Toma, C, Tokgoz Akyil, F, Tobino, K, Tobar, R, Tiwari, A, Tibana, R, Tian, X, Thillai, M, Tham, W, Teo, F, Tekavec Trkanjec, J, Teixeira, P, Tarpey, D, Tapias, L, Tanizawa, K, Tanino, Y, Takada, T, Tabaj, G, Szolnoki, E, Swarnakar, R, Strambu, I, Sterclova, M, Spinks, K, Soo, C, Soltani, A, Solanki, S, Sobh, E, Soares, M, Smith, J, Smith, B, Slocum, P, Slabbynck, H, Sivokozov, I, Shifren, A, Shen, S, Sharp, C, Shanmuganathan, A, Sebastiani, A, Scarlata, S, Savas, R, Sasaki, S, Santeliz, J, Santana, A, Sanchez, R, Salinas, M, Saito, S, Ryan, F, Royo Prats, J, Rosi, E, Rokadia, H, Robles Perez, A, Rivera Ortega, P, Rio Ramirez, M, Righetti, S, Reichner, C, Ravaglia, C, Ratanawatkul, P, Ramalingam, V, Rajasekaran, A, Radzikowska, E, Ra, S, Quadrelli, S, Precerutti, J, Prasad, J, Popa, D, Pizzalato, S, Piotrowski, W, Pineiro, A, Piloni, D, Peros Golubicic, T, Perez, R, Pereira, C, Pereira, B, Perch, M, Patel, N, Patel, D, Papanikolaou, I, Papakosta, D, Panselinas, E, Pang, Y, Pandya, P, Padrao, E, Ozdemir Kumbasar, O, Overbeek, M, Otto Minasian, A, O'Riordan, D, Ora, J, Oldham, J, Okutan, O, Ohshimo, S, Oguzulgen, I, Ogura, T, O'Donnell, T, O'Dochartaigh, C, O'Beirne, S, Novikova, L, Novelli, L, Noth, I, Nogueira Mendes Neto, N, Niroumand, M, Nieto, A, Neves, A, Nambiar, A, Nair, S, Nadama, R, Murtagh, E, Mura, M, Muller Quernheim, J, Mukhopadhyay, A, Mukherjee, S, Morisset, J, Moran, O, Mooney, J, Moller, J, Mogulkoc, N, Miyamoto, A, Milenkovic, B, Mette, S, Mejia, M, Mei, F, Mazzei, M, Matsuda, T, Mason, C, Martinez Frances, M, Mannarino, S, Mancuzo, E, Malli, F, Malhotra, P, Maillo, M, Maia, J, Mahdavian, M, Madsen, F, Luckhardt, T, Lucht, W, Low, S, Lopez Miguel, C, Lipchik, R, Levy, S, Levin, K, Lee, K, Lederer, D, Lammi, M, Kwan, H, Kukreja, S, Kruavit, A, Kotecki, M, Kolilekas, L, Knoop, H, Kiyan, E, Kishaba, T, King Biggs, M, Khor, Y, Khan, A, Khalil, N, Kedia, R, Kebba, N, Kawano Dourado, L, Kapitan, K, Kan, C, Kalyoncu, A, Kalluri, M, Kabasakal, Y, Jyothula, S, Juretschke, M, Jovanovic, D, Jonkers, R, Jo, H, Izumi, S, Ishii, H, Ikeda, S, Ibrahim, A, Hyldgaard, C, Hunninghake, G, Huie, T, Hufton, A, Hu, X, Hseih, W, Hoyos, R, Hoyles, R, Holguin Rodriguez, O, Hogan, M, Hodgson, U, Hilkin Sogoloff, H, Herrera, E, Henry, B, Hellemons, M, Hecimovic, A, Hayashi, R, Hart, S, Harari, S, Haney, S, Hambly, N, Hakkim, R, Gutierrez, M, Gripaldo, R, Gomez, A, Goh, N, Godoy, R, Gilbert, C, Giannarakis, I, Gasparini, S, Garcha, P, Furtado, S, Fois, A, Flood Page, P, Fletcher, S, Fiss, E, Figueroa Casas, J, Figueroa Casas, M, Fiddler, C, Ferrara, G, Fernandez Casares, M, Felton, C, Faverio, P, Fabro, A, Estrada, A, Errhalt, P, Enomoto, N, Enghelmayer, J, El Kersh, K, Eiger, G, Dubaniewicz, A, Drakopanagiotakis, F, Disayabutr, S, Dijkstra, A, Diaz Patino, J, Diaz Castanon, J, Dhooria, S, Dhasmana, D, De Rosa, M, De Luca, S, Delobbe, A, Delgado, D, Delgado, C, De La Fuente, I, De Kruif, M, De Gier, M, De Andrade, J, Davidsen, J, Daoud, B, Dalhoff, K, Cotera Solano, J, Costa, A, Coronel, S, Confalonieri, M, Conemans, L, Comellas, A, Colella, S, Clemente, S, Clark, J, Ciuffreda, M, Chung, C, Chong, S, Chirita, D, Chen, P, Chaudhuri, N, Chambers, D, Chalmers, G, Chairman, D, Chai, G, Chacon Chaves, R, Cetinsu, V, Ceruti, M, Ceballos Zuniga, C, Castillo, D, Carbone, R, Caminati, A, Callejas Gonzalez, F, Butler, M, Bustos, C, Bukowczan, M, Buendia, I, Brunetti, G, Brockway, B, Bresser, P, Breseghello, J, Bouros, D, Botero Zaccour, J, Borzone, G, Borie, R, Blum, H, Blank, J, Biswas, A, Bennett, D, Benjamin, M, Belaconi, I, Beirne, P, Beckert, L, Bastiampillai, S, Bascom, R, Bartholmai, B, Barros, M, Ban, A, Balestro, E, Baldi, B, Baddini Martinez, J, Baburao, A, Babu, S, Averyanov, A, Avdeev, S, Athanazio, R, Atahan, E, Asuquo, B, Assayag, D, Antuni, J, Antillon, S, Anderson, K, Anderson, A, Alwani, F, Altinisik, G, Alsouofi, N, Allam, J, Al Jahdali, H, Al Farttoosi, A, Alfaro, T, Al Busaidi, N, Alavi Foumani, A, Agreda Vedia, M, Agarwal, A, Afridi, F, Adeyeye, O, Adegunsoye, A, Adamali, H, Abedini, A, and Pulmonary Medicine

Subjects
Male, Pediatrics, International Cooperation, Respiratory System, Hospitals, University, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Cohen's kappa, Diagnosis, UK, 030212 general & internal medicine, Medical diagnosis, Referral and Consultation, Pulmonologists, Idiopathic Pulmonary Fibrosi, Interstitial lung disease, 11 Medical And Health Sciences, Middle Aged, respiratory system, Prognosis, Hospitals, humanities, Dimensional Measurement Accuracy, Clinical Competence, Diagnosis, Differential, Diagnostic Techniques, Respiratory System, Female, Humans, Idiopathic Pulmonary Fibrosis, Quality of Health Care, Reproducibility of Results, Human, Cohort study, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Prognosi, education, MEDLINE, Reproducibility of Result, INTERSTITIAL PNEUMONIA, Interstitial Lung Diseases, 03 medical and health sciences, Internal medicine, PARENCHYMAL LUNG-DISEASE, MANAGEMENT, medicine, Idiopathic pulmonary fibrosis, diagnosis, Pulmonologist, University, business.industry, MORTALITY, Original Articles, medicine.disease, respiratory tract diseases, Diagnostic Techniques, IPF Project Consortium, 030228 respiratory system, Differential, INTEROBSERVER AGREEMENT, UPDATE, COOPERAÇÃO INTERNACIONAL, Differential diagnosis, business
Abstract

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts., Academic status, access to MDT meetings and clinician experience predict accuracy of a clinical diagnosis of IPF http://ow.ly/k43W30cTMg1

Published
2017
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14. Severe asthma: Entering an era of new concepts and emerging therapies: Highlights of the 4th international severe asthma forum, Madrid, 2018

Author

Seys, S.F. Quirce, S. Agache, I. Akdis, C.A. Alvaro-Lozano, M. Antolín-Amérigo, D. Bjermer, L. Bobolea, I. Bonini, M. Bossios, A. Brinkman, P. Bush, A. Calderon, M. Canonica, W. Chanez, P. Couto, M. Davila, I. Del Giacco, S. Del Pozo, V. Erjefält, J.S. Gevaert, P. Hagedoorn, P. G. Heaney, L. Heffler, E. Hellings, P.W. Jutel, M. Kalayci, O. Kurowski, M.M. Loukides, S. Nair, P. Palomares, O. Polverino, E. Sanchez-Garcia, S. Sastre, J. Schwarze, J. Spanevello, A. Ulrik, C.S. Usmani, O. Van den Berge, M. Vasakova, M. Vijverberg, S. Diamant, Z.

Published
2019

15. Analysis of Effectiveness of Pirfenidone in Subgroups of Idiopathic Pulmonary Fibrosis Based on Real World Data from European IPF Registry

Author

Krauss, E., primary, Drakopanagiotakis, F., additional, Costabel, U., additional, Nieto Barbero, M.A., additional, Mueller, V., additional, Bonniaud, P., additional, Vancheri, C., additional, Wells, A.U., additional, Vasakova, M., additional, Pesci, A., additional, Klepetko, W., additional, Seeger, W., additional, Crestani, B., additional, and Guenther, A., additional

Published
2019
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17. Long-Term Safety and Efficacy of Recombinant Human Pentraxin-2 in Patients with Idiopathic Pulmonary Fibrosis

Author

Raghu, G., primary, van den Blink, B., additional, Hamblin, M.J., additional, Brown, A.W., additional, Golden, J.A., additional, Ho, L.A., additional, Wijsenbeek, M.S., additional, Vasakova, M., additional, Pesci, A., additional, Antin-Ozerkis, D.E., additional, Meyer, K., additional, Kreuter, M., additional, Santin-Janin, H., additional, Aubin, F., additional, Mulder, G.-J.J., additional, Gupta, R., additional, and Richeldi, L., additional

Published
2019
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18. Serious adverse events in patients with idiopathic pulmonary fibrosis in the placebo arms of 6 clinical trials

Author

Wuyts, W., Antin-Ozerkis, D., Huggins, J.T., LaCamera, P.P., Spagnolo, P. (Paolo), Vasakova, M., Wijsenbeek-Lourens, M.S., Polman, B., Kirchgaessler, K. U., Scholand, M.B., Wuyts, W., Antin-Ozerkis, D., Huggins, J.T., LaCamera, P.P., Spagnolo, P. (Paolo), Vasakova, M., Wijsenbeek-Lourens, M.S., Polman, B., Kirchgaessler, K. U., and Scholand, M.B.

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease characterized by irreversible loss of lung function and an unpredictable course of disease progression. Methods: The safety data for patients with IPF who received placebo in 6 clinical trials were pooled to examine the categories and frequencies of serious adverse events (SAEs) in this population. Results: In 1082 patients with IPF who received placebo, 673 SAEs were reported. Of these, 93 SAEs resulted in death (8.6% of patients). Respiratory-related conditions were the most frequently reported SAE (225 events, 16.33 per 100 patient-exposure years [PEY]), followed by infections and infestations (136 events, 9.87 per 100 PEY) and cardiac disorders (79 events, 5.73 per 100 PEY); these categories also had the most fatal outcomes (60, 10, and 10 deaths, respectively). The most frequently reported fatal respiratoryrelated SAEs were IPF and respiratory failure (38 and 11 patients, respectively), and the most frequently reported fatal infections and infestations and cardiac disorders were pneumonia (5 patients) and myocardial infarcti

Published
2019
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19. Psychometric properties and minimal important differences of SF-36 in Idiopathic Pulmonary Fibrosis

Author

Witt, S, Krauss, E, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Klepetko, W, Seeger, W, Crestani, B, Leidl, R, Holle, R, Schwarzkopf, L, Guenther, A, Witt, Sabine, Krauss, Ekaterina, Barbero, María Asunción Nieto, Müller, Veronika, Bonniaud, Philippe, Vancheri, Carlo, Wells, Athol U, Vasakova, Martina, Pesci, Alberto, Klepetko, Walter, Seeger, Werner, Crestani, Bruno, Leidl, Reiner, Holle, Rolf, Schwarzkopf, Larissa, Guenther, Andreas, Witt, S, Krauss, E, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Klepetko, W, Seeger, W, Crestani, B, Leidl, R, Holle, R, Schwarzkopf, L, Guenther, A, Witt, Sabine, Krauss, Ekaterina, Barbero, María Asunción Nieto, Müller, Veronika, Bonniaud, Philippe, Vancheri, Carlo, Wells, Athol U, Vasakova, Martina, Pesci, Alberto, Klepetko, Walter, Seeger, Werner, Crestani, Bruno, Leidl, Reiner, Holle, Rolf, Schwarzkopf, Larissa, and Guenther, Andreas

Abstract

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a rare disease with a median survival of 3-5 years after diagnosis with limited treatment options. The aim of this study is to assess the psychometric characteristics of the Short Form 36 Health Status Questionnaire (SF-36) in IPF and to provide disease specific minimally important differences (MID). METHODS: Data source was the European IPF Registry (eurIPFreg). The psychometric properties of the SF-36 version 2 were evaluated based on objective clinical measures as well as subjective perception. We analysed acceptance, feasibility, discrimination ability, construct and criterion validity, responsiveness and test-retest-reliability. MIDs were estimated via distribution and anchor-based approaches. RESULTS: The study population included 258 individuals (73.3% male; mean age 67.3 years, SD 10.7). Of them 75.2% (194 individuals) had no missing item. The distribution of several items was skewed, although floor effect was acceptable. Physical component score (PCS) correlated significantly and moderately with several anchors, whereas the correlations of mental component score (MCS) and anchors were only small. The tests showed mainly significant lower HRQL in individuals with long-term oxygen therapy. Analyses in stable individuals did not show significant changes of HRQL except for one dimension and anchor. Individuals with relevant changes of the health status based on the anchors had significant changes in all SF-36 dimensions and summary scales except for the dimension PAIN. PCS and MCS had mean MIDs of five and six, respectively. Mean MIDs of the dimensions ranged from seven to 21. CONCLUSION: It seems that the SF-36 is a valid instrument to measure HRQL in IPF and so can be used in RCTs or individual monitoring of disease. Nevertheless, the additional evaluation of longitudinal aspects and MIDs can be recommended to further analyse these factors. Our findings have a great potential impact on the evaluation of IPF p

Published
2019

20. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study

Author

Raghu, G, van den Blink, B, Hamblin, M, Brown, A, Golden, J, Ho, L, Wijsenbeek, M, Vasakova, M, Pesci, A, Antin-Ozerkis, D, Meyer, K, Kreuter, M, Moran, D, Santin-Janin, H, Aubin, F, Mulder, G, Gupta, R, Richeldi, L, Raghu, Ganesh, van den Blink, Bernt, Hamblin, Mark J, Brown, A Whitney, Golden, Jeffrey A, Ho, Lawrence A, Wijsenbeek, Marlies S, Vasakova, Martina, Pesci, Alberto, Antin-Ozerkis, Danielle E, Meyer, Keith C, Kreuter, Michael, Moran, Donna, Santin-Janin, Hugues, Aubin, Francois, Mulder, Geert-Jan, Gupta, Renu, Richeldi, Luca, Raghu, G, van den Blink, B, Hamblin, M, Brown, A, Golden, J, Ho, L, Wijsenbeek, M, Vasakova, M, Pesci, A, Antin-Ozerkis, D, Meyer, K, Kreuter, M, Moran, D, Santin-Janin, H, Aubin, F, Mulder, G, Gupta, R, Richeldi, L, Raghu, Ganesh, van den Blink, Bernt, Hamblin, Mark J, Brown, A Whitney, Golden, Jeffrey A, Ho, Lawrence A, Wijsenbeek, Marlies S, Vasakova, Martina, Pesci, Alberto, Antin-Ozerkis, Danielle E, Meyer, Keith C, Kreuter, Michael, Moran, Donna, Santin-Janin, Hugues, Aubin, Francois, Mulder, Geert-Jan, Gupta, Renu, and Richeldi, Luca

Abstract

Background: Patients with idiopathic pulmonary fibrosis (IPF) treated with PRM-151, a recombinant human pentraxin 2 protein, in a phase 2 double-blind, randomised controlled trial had significantly reduced decline in percentage of predicted forced vital capacity (FVC) and stabilised 6-min walking distance compared with placebo over a 28-week period. Here we report the 76-week results of an open-label extension study. Methods: Patients who completed the 28-week double-blind period of the PRM-151-202 trial were eligible to participate in the open-label extension study. Patients previously enrolled in the PRM-151 group continued this treatment and those previously in the placebo group crossed over to PRM-151. All patients received PRM-151 in 28-week cycles with loading doses of 10 mg/kg by 60 min intravenous infusions on days 1, 3, and 5 in the first week of each cycle followed by one infusion of 10 mg/kg every 4 weeks. The primary objective of the open-label extension study was to assess the long-term safety and tolerability of PRM-151, which were assessed by analysing adverse events (AEs) up to week 76 in all patients who received at least one dose of PRM-151 during the open-label extension study. Exploratory efficacy analyses were done by assessing changes from baseline in percentage of predicted FVC and 6-min walking distance, with descriptive statistics to week 76 and with random-intercept mixed models to week 52. This study is registered with ClinicalTrials.gov, number NCT02550873, and with EudraCT, number 2014-004782-24. Findings: Of 116 patients who completed the double-blind treatment period, 111 entered the open-label extension study (74 from the PRM-151 group and 37 from the placebo group). 84 (76%) of 111 patients received concomitant IPF therapy (pirfenidone n=55 or nintedanib n=29). AEs were consistent with long-term IPF sequelae. 31 (28%) patients had serious AEs. Those occurring in two or more patients were pneumonia (six [5%] of 111), IPF exacerbation

Published
2019

22. Severe asthma: Entering an era of new concepts and emerging therapies: Highlights of the 4th international severe asthma forum, Madrid, 2018

Author

Seys, S. F., Quirce, S., Agache, I., Akdis, C. A., Alvaro-Lozano, M., Antolin-Amerigo, D., Bjermer, L., Bobolea, I., Bonini, M., Bossios, A., Brinkman, P., Bush, A., Calderon, M., Canonica, W., Chanez, P., Couto, M., Davila, I., Del Giacco, S., Del Pozo, V., Erjefalt, J. S., Gevaert, P., Hagedoorn, P., G. Heaney, L., Heffler, E., Hellings, P. W., Jutel, M., Kalayci, O., Kurowski, M. M., Loukides, S., Nair, P., Palomares, O., Polverino, E., Sanchez-Garcia, S., Sastre, J., Schwarze, J., Spanevello, A., Ulrik, C. S., Usmani, O., Van den Berge, M., Vasakova, M., Vijverberg, S., Diamant, Z., Bonini M. (ORCID:0000-0002-3042-0765), Seys, S. F., Quirce, S., Agache, I., Akdis, C. A., Alvaro-Lozano, M., Antolin-Amerigo, D., Bjermer, L., Bobolea, I., Bonini, M., Bossios, A., Brinkman, P., Bush, A., Calderon, M., Canonica, W., Chanez, P., Couto, M., Davila, I., Del Giacco, S., Del Pozo, V., Erjefalt, J. S., Gevaert, P., Hagedoorn, P., G. Heaney, L., Heffler, E., Hellings, P. W., Jutel, M., Kalayci, O., Kurowski, M. M., Loukides, S., Nair, P., Palomares, O., Polverino, E., Sanchez-Garcia, S., Sastre, J., Schwarze, J., Spanevello, A., Ulrik, C. S., Usmani, O., Van den Berge, M., Vasakova, M., Vijverberg, S., Diamant, Z., and Bonini M. (ORCID:0000-0002-3042-0765)

Abstract

N.D.

Published
2019

23. Validation of multidisciplinary diagnosis in IPF

Author

Castillo, D., Walsh, S., Hansell, D.M., Vasakova, M., Cottin, V., Altınışık, Göksel, Palmucci, S., Sterclova, M., Harari, S., Richeldi, L., Vancheri, C., and Wells, A.U.

Subjects
survival rate, multidisciplinary diagnosis, antifibrotic agent, clinical examination, follow up, Humans, diagnostic test accuracy study, human, survival time, reproducibility, interstitial pneumonia, standardization, Patient Care Team, allergic pneumonitis, medical specialist, practice guideline, clinical audit, patient care, consensus development, standard, Reproducibility of Results, Note, Idiopathic Pulmonary Fibrosis, clinical practice, fibrosing alveolitis, priority journal, validation study, disease exacerbation, Practice Guidelines as Topic, diagnostic accuracy, diagnostic value, prognosis, teamwork, clinical competence
Published
2018

24. The European IPF registry (eurIPFreg): Baseline characteristics and survival of patients with idiopathic pulmonary fibrosis

Author

Guenther, A, Krauss, E, Tello, S, Wagner, J, Paul, B, Kuhn, S, Maurer, O, Heinemann, S, Costabel, U, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Sofia, M, Klepetko, W, Seeger, W, Drakopanagiotakis, F, Crestani, B, Guenther, Andreas, Krauss, Ekaterina, Tello, Silke, Wagner, Jasmin, Paul, Bettina, Kuhn, Stefan, Maurer, Olga, Heinemann, Sabine, Costabel, Ulrich, Barbero, María Asunción Nieto, Müller, Veronika, Bonniaud, Philippe, Vancheri, Carlo, Wells, Athol, Vasakova, Martina, Pesci, Alberto, Sofia, Matteo, Klepetko, Walter, Seeger, Werner, Drakopanagiotakis, Fotios, Crestani, Bruno, Guenther, A, Krauss, E, Tello, S, Wagner, J, Paul, B, Kuhn, S, Maurer, O, Heinemann, S, Costabel, U, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Sofia, M, Klepetko, W, Seeger, W, Drakopanagiotakis, F, Crestani, B, Guenther, Andreas, Krauss, Ekaterina, Tello, Silke, Wagner, Jasmin, Paul, Bettina, Kuhn, Stefan, Maurer, Olga, Heinemann, Sabine, Costabel, Ulrich, Barbero, María Asunción Nieto, Müller, Veronika, Bonniaud, Philippe, Vancheri, Carlo, Wells, Athol, Vasakova, Martina, Pesci, Alberto, Sofia, Matteo, Klepetko, Walter, Seeger, Werner, Drakopanagiotakis, Fotios, and Crestani, Bruno

Abstract

Background: Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research. Methods: The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management. Results: This paper describes baseline data of 525 IPF subjects recruited from 11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value, DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under which patients showed improved survival (p = 0.001). Conclusions: Our data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time.

Published
2018

25. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis a randomized clinical trial

Author

Raghu, G, Van Den Blink, B, Hamblin, M, Whitney Brown, A, Golden, J, Ho, L, Wijsenbeek, M, Vasakova, M, Pesci, A, Antin-Ozerkis, D, Meyer, K, Kreuter, M, Santin-Janin, H, Mulder, G, Bartholmai, B, Gupta, R, Richeldi, L, Raghu, Ganesh, Van Den Blink, Bernt, Hamblin, Mark J., Whitney Brown, A., Golden, Jeffrey A., Ho, Lawrence A., Wijsenbeek, Marlies S., Vasakova, Martina, Pesci, Alberto, Antin-Ozerkis, Danielle E., Meyer, Keith C., Kreuter, Michael, Santin-Janin, Hugues, Mulder, Geert-Jan, Bartholmai, Brian, Gupta, Renu, Richeldi, Luca, Raghu, G, Van Den Blink, B, Hamblin, M, Whitney Brown, A, Golden, J, Ho, L, Wijsenbeek, M, Vasakova, M, Pesci, A, Antin-Ozerkis, D, Meyer, K, Kreuter, M, Santin-Janin, H, Mulder, G, Bartholmai, B, Gupta, R, Richeldi, L, Raghu, Ganesh, Van Den Blink, Bernt, Hamblin, Mark J., Whitney Brown, A., Golden, Jeffrey A., Ho, Lawrence A., Wijsenbeek, Marlies S., Vasakova, Martina, Pesci, Alberto, Antin-Ozerkis, Danielle E., Meyer, Keith C., Kreuter, Michael, Santin-Janin, Hugues, Mulder, Geert-Jan, Bartholmai, Brian, Gupta, Renu, and Richeldi, Luca

Abstract

IMPORTANCE Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with poor prognosis. Approved therapies do not halt disease progression. OBJECTIVE To determine the effect of recombinant human pentraxin 2 vs placebo on change from baseline to week 28 in mean forced vital capacity (FVC) percentage of predicted value. DESIGN, SETTING, AND PARTICIPANTS Phase 2, randomized, double-blind, placebo-controlled trial conducted at 18 sites in 7 countries of eligible patients with IPF (N = 117; aged 40-80 years; FVC 50% and 90% predicted; ratio of forced expiratory volume in the first second/FVC >0.70; diffusing capacity for carbon monoxide [DLCO] 25% and 90% predicted; and distance of 150 m on the 6-minute walk test). Study period was August 2015-May 2017. INTERVENTIONS Patients were randomized to receive either recombinant human pentraxin 2 (10 mg/kg intravenous every 4 weeks, n = 77) or placebo (n = 39) for 24 weeks, and stratified by concurrent IPF treatment status. MAIN OUTCOMES AND MEASURES The primary end point was the least-squares mean change in FVC percentage of predicted value from baseline to week 28 (minimal clinically important difference, decline of 2%-6%). Secondary end points included mean change in lung volumes (total, normal, and interstitial lung abnormalities) on high-resolution computed tomography (HRCT) and 6-minute walk distance (minimal clinically important difference, 24-45 m). RESULTS Of 117 randomized patients, 116 received at least 1 dose of study drug (mean age, 68.6 years; 81.0% men; mean time since IPF diagnosis, 3.8 years), and 111 (95.7%) completed the study. The least-squares mean change in FVC percentage of predicted value from baseline to week 28 in patients treated with recombinant human pentraxin 2 was -2.5 vs -4.8 for those in the placebo group (difference, +2.3 [90% CI, 1.1 to 3.5]; P = .001). No significant treatment differences were observed in total lung volume (difference, 93.5 mL [90% CI, -27.7 to 214.7]), quan

Published
2018

26. Antacid therapy in idiopathic pulmonary fibrosis: more questions than answers?

Author

Johannson, K, Strâmbu, I, Ravaglia, C, Grutters, J, Valenzuela, C, Mogulkoc, N, Luppi, F, Richeldi, L, Wells, A, Vancheri, C, Kreuter, M, Albera, C, Antoniou, K, Altinisik, G, Bendstrup, E, Bondue, B, Borie, R, Brown, K, Camus, P, Castillo, D, Collard, H, Cottin, V, Crimi, N, Ferrara, G, Fischer, A, Gauldie, J, Geiser, T, Guenther, A, Hambly, N, Hansell, D, Harari, S, Jones, M, Keane, M, Ley, B, Maher, T, Molina-Molina, M, Palmucci, S, Poletti, V, Prasse, A, Rottoli, P, Spagnolo, P, Sterclova, M, Torrisi, S, Tsitoura, E, Vasakova, M, Walsh, S, Wijsenbeek, M, Wuyts, W, Johannson, Kerri A, Strâmbu, Irina, Ravaglia, Claudia, Grutters, Jan C, Valenzuela, Claudia, Mogulkoc, Nesrin, Luppi, Fabrizio, Richeldi, Luca, Wells, Athol U, Vancheri, Carlo, Kreuter, Michael, Albera, Carlo, Antoniou, Katerina M., Altinisik, Goksel, Bendstrup, Elisabeth, Bondue, Benjamin, Borie, Raphael, Brown, Kevin K., Camus, Philippe, Castillo, Diego, Collard, Harold R., Cottin, Vincent, Crimi, Nunzio, Ferrara, Giovanni, Fischer, Aryeh, Gauldie, Jack, Geiser, Thomas, Guenther, Andreas, Hambly, Nathan, Hansell, David M., Harari, Sergio, Jones, Mark G., Keane, Michael, Ley, Brett, Maher, Toby M., Molina-Molina, Maria, Palmucci, Stefano, Poletti, Venerino, Prasse, Antje, Rottoli, Paola, Spagnolo, Paolo, Sterclova, Martina, Torrisi, Sebastiano, Tsitoura, Eliza, Vasakova, Martina, Walsh, Simon L., Wijsenbeek, Marlies S., Wuyts, Wim A., Johannson, K, Strâmbu, I, Ravaglia, C, Grutters, J, Valenzuela, C, Mogulkoc, N, Luppi, F, Richeldi, L, Wells, A, Vancheri, C, Kreuter, M, Albera, C, Antoniou, K, Altinisik, G, Bendstrup, E, Bondue, B, Borie, R, Brown, K, Camus, P, Castillo, D, Collard, H, Cottin, V, Crimi, N, Ferrara, G, Fischer, A, Gauldie, J, Geiser, T, Guenther, A, Hambly, N, Hansell, D, Harari, S, Jones, M, Keane, M, Ley, B, Maher, T, Molina-Molina, M, Palmucci, S, Poletti, V, Prasse, A, Rottoli, P, Spagnolo, P, Sterclova, M, Torrisi, S, Tsitoura, E, Vasakova, M, Walsh, S, Wijsenbeek, M, Wuyts, W, Johannson, Kerri A, Strâmbu, Irina, Ravaglia, Claudia, Grutters, Jan C, Valenzuela, Claudia, Mogulkoc, Nesrin, Luppi, Fabrizio, Richeldi, Luca, Wells, Athol U, Vancheri, Carlo, Kreuter, Michael, Albera, Carlo, Antoniou, Katerina M., Altinisik, Goksel, Bendstrup, Elisabeth, Bondue, Benjamin, Borie, Raphael, Brown, Kevin K., Camus, Philippe, Castillo, Diego, Collard, Harold R., Cottin, Vincent, Crimi, Nunzio, Ferrara, Giovanni, Fischer, Aryeh, Gauldie, Jack, Geiser, Thomas, Guenther, Andreas, Hambly, Nathan, Hansell, David M., Harari, Sergio, Jones, Mark G., Keane, Michael, Ley, Brett, Maher, Toby M., Molina-Molina, Maria, Palmucci, Stefano, Poletti, Venerino, Prasse, Antje, Rottoli, Paola, Spagnolo, Paolo, Sterclova, Martina, Torrisi, Sebastiano, Tsitoura, Eliza, Vasakova, Martina, Walsh, Simon L., Wijsenbeek, Marlies S., and Wuyts, Wim A.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive parenchymal lung disease of complex cause. Gastro-oesophageal reflux (GER) and microaspiration have been proposed as risk factors for the development and progression of IPF, but robust definitive data are few. A recent international guideline conditionally recommended the use of antacid therapy (proton pump inhibitors or histamine-2-receptor antagonists) for patients with IPF, in the absence of oesophageal reflux or symptoms. In this Position Paper, we summarise the literature addressing the association between GER and IPF, and also identify future research priorities that could clarify this issue. We shed light on the process through which the guideline recommendation was achieved and aim to contextualise the recommendation for providers caring for patients with IPF.

Published
2017

28. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis

Author

Luca, Richeldi, du Bois, Roland M., Ganesh, Raghu, Arata, Azuma, Brown, Kevin K., Ulrich, Costabel, Vincent, Cottin, Flaherty, Kevin R., Hansell, David M., Yoshikazu, Inoue, Dong Soon Kim, Martin, Kolb, Nicholson, Andrew G., Noble, Paul W., Moisés, Selman, Hiroyuki, Taniguchi, Michèle, Brun, Florence Le Maulf, Mannaïg, Girard, Susanne, Stowasser, Rozsa Schlenker Herceg, Bernd, Disse, Collard, Harold R., Corte, T, Davies, H, Glaspole, I, Mulder, J, Veitch, E, De Vuyst, P, Liistro, G, Sibille, Y, Vincken, W, Wuyts, W, Fell, C, Hernandez, P, Kolb, M, Undurraga, A, Bai, C, Chen, P, Gao, Z, Kang, J, Li, H, Li, Z, Wan, H, Wang, H, Wen, F, Xiao, Q, Xu, Z, Zhang, W, Zheng, X, Zhu, H, Pauk, N, Reiterer, P, Vasakova, M, Hodgson, U, Bourdin, A, Cadranel, J, Camus, P, Chanez, P, Cottin, V, Crestani, B, Israel Biet, D, Jouneau, S, Lebargy, F, Marquette, C, Prévot, G, Valeyre, D, Wallaert, B, Bonnet, R, Costabel, U, Gläser, S, Grohé, C, Guenther, A, Hammerl, P, Höffken, G, Karagiannidis, C, Kirschner, J, Kirsten, A, Korn, S, Kreuter, M, Müller Quernheim, J, Neurohr, C, Pfeifer, M, Schönfeld, N, Wiewrodt, R, Antoniou, K, Daniil, Z, Diamantea, F, Koulouris, N, Mathioudakis, G, Ghosal, A, Kadappa Shivappa, S, Kawedia, M, Khatavkar, P, Kumar, A, Mehta, P, Singh, V, Srikanth, K, Thakker, H, Udwadia, Z, Egan, J, Fink, G, Kramer, M, Yigla, M, Agostini, Carlo, De Benedetto, F, Harari, S, Luppi, F, Paggiaro, P, Tavanti, L, Pesci, A, Poletti, V, Rottoli, P, Saltini, C, Sanduzzi Zamparelli, A, Vancheri, C, Bando, M, Hasegawa, Y, Hashimoto, K, Homma, S, Inase, N, Inoue, Y, Arai, T, Izumi, S, Kawamura, T, Kishi, K, Kondo, Y, Kuwano, K, Miura, Y, Nishioka, Y, Nishiyama, O, Ogura, T, Ohkouchi, S, Saito, T, Setoguchi, Y, Shindoh, J, Taguchi, Y, Tanakadate, M, Tomii, K, Sugita, Y, Yamaguchi, T, Yoshimori, K, Jeong, S, Kim, D, Kim, Y, Park, C, Song, J, Uh, S, Selman, M, Bresser, P, Grutters, J, Wijsenbeek, M, Arrobas, A, Cardoso, J, Costa, R, Morais, A, Neves, S, Serrado, M, Ilkovick, M, Vizel, A, Alfageme Michavila, I, Ancochea, J, Castillo Villegas, D, Molina Molina, M, Morell, F, Xaubet, A, Aktogu Ozkan, S, Kayacan, O, Ongen, G, Mogulkoc, N, Tuncay, E, Beirne, P, Bettinson, H, Burge, P, Dempsey, O, Maher, T, Millar, A, Spencer, L, Thickett, D, Alvarez, J, Andrews, C, Bajwa, O, Baker, A, Baughman, R, Belperio, J, Bradley, J, Collard, H, Cordova, F, Daniels, C, de Andrade, J, Dushay, K, Enelow, R, Ettinger, N, Gibson, K, Gotfried, M, Hajari Case, A, Hotchkin, D, Huggins, J, Kaye, M, Kershaw, C, Kureishy, S, Lancaster, L, Lederer, D, Mageto, Y, Masson, J, Meyer, K, Mohabir, P, Morrison, L, Nathan, S, Noth, I, Oelberg, D, Rahaghi, F, Riley, D, Rizzo, A, Rossman, M, Ruzi, J, Sachs, P, Schaumberg, T, Scholand, M, Schroeder, C, Seifer, F, Shea, J, Sinkowitz, D, Tabak, J, Taylor, J, Thompson, J, Thurm, C, Tita, J, Wencel, M, Westerman, J, Lasky, J, Demedts, M, Casteels, M, Loddenkemper, R, Michaelis, J, Roman, J, Tino, G, Luisetti, M., and Clinical sciences

Subjects
Male, medicine.medical_specialty, Vital capacity, Indoles, Exacerbation, Aged, Disease Progression, Double-Blind Method, Enzyme Inhibitors, Female, Humans, Idiopathic Pulmonary Fibrosis, Middle Aged, Protein Kinase Inhibitors, Protein-Tyrosine Kinases, Quality of Life, Treatment Outcome, Vital Capacity, Settore MED/10 - Malattie dell'Apparato Respiratorio, Medizin, Placebo, Gastroenterology, FEV1/FVC ratio, Idiopathic pulmonary fibrosis, chemistry.chemical_compound, Internal medicine, medicine, business.industry, Medicine (all), Hazard ratio, General Medicine, Pirfenidone, medicine.disease, Surgery, chemistry, Nintedanib, business, medicine.drug
Abstract

Background Nintedanib (formerly known as BIBF 1120) is an intracellular inhibitor that targets multiple tyrosine kinases. A phase 2 trial suggested that treatment with 150 mg of nintedanib twice daily reduced lung-function decline and acute exacerbations in patients with idiopathic pulmonary fibrosis. Methods We conducted two replicate 52-week, randomized, double-blind, phase 3 trials (INPULSIS-1 and INPULSIS-2) to evaluate the efficacy and safety of 150 mg of nintedanib twice daily as compared with placebo in patients with idiopathic pulmonary fibrosis. The primary end point was the annual rate of decline in forced vital capacity (FVC). Key secondary end points were the time to the first acute exacerbation and the change from baseline in the total score on the St. George’s Respiratory Questionnaire, both assessed over a 52-week period. Results A total of 1066 patients were randomly assigned in a 3:2 ratio to receive nintedanib or placebo. The adjusted annual rate of change in FVC was −114.7 ml with nintedanib versus −239.9 ml with placebo (difference, 125.3 ml; 95% confidence interval [CI], 77.7 to 172.8; P

Published
2014

38. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study

Author

A. Whitney Brown, Renu Gupta, Ganesh Raghu, Luca Richeldi, Mark J. Hamblin, Lawrence A. Ho, Marlies S. Wijsenbeek, Bernt van den Blink, Jeffrey A. Golden, Hugues Santin-Janin, Michael Kreuter, Geert-Jan Mulder, Martina Vasakova, Danielle Antin-Ozerkis, Francois Aubin, Keith C. Meyer, Donna Moran, Alberto Pesci, Raghu, G, van den Blink, B, Hamblin, M, Brown, A, Golden, J, Ho, L, Wijsenbeek, M, Vasakova, M, Pesci, A, Antin-Ozerkis, D, Meyer, K, Kreuter, M, Moran, D, Santin-Janin, H, Aubin, F, Mulder, G, Gupta, R, Richeldi, L, and Pulmonary Medicine

Subjects
Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Exacerbation, Vital Capacity, Settore MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, Placebo, law.invention, 03 medical and health sciences, FEV1/FVC ratio, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Double-Blind Method, Randomized controlled trial, law, Internal medicine, Humans, Medicine, 030212 general & internal medicine, Extensive stage, Aged, idiopathic pulmonary fibrosis, pentraxin 2, Homeodomain Proteins, Cross-Over Studies, MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, business.industry, medicine.disease, Long-Term Care, Crossover study, Idiopathic Pulmonary Fibrosis, Recombinant Proteins, Serum Amyloid P-Component, Treatment Outcome, 030228 respiratory system, Tolerability, Female, idiopathic pulmonary fibrosis (IPF), business
Abstract

Background: Patients with idiopathic pulmonary fibrosis (IPF) treated with PRM-151, a recombinant human pentraxin 2 protein, in a phase 2 double-blind, randomised controlled trial had significantly reduced decline in percentage of predicted forced vital capacity (FVC) and stabilised 6-min walking distance compared with placebo over a 28-week period. Here we report the 76-week results of an open-label extension study. Methods: Patients who completed the 28-week double-blind period of the PRM-151-202 trial were eligible to participate in the open-label extension study. Patients previously enrolled in the PRM-151 group continued this treatment and those previously in the placebo group crossed over to PRM-151. All patients received PRM-151 in 28-week cycles with loading doses of 10 mg/kg by 60 min intravenous infusions on days 1, 3, and 5 in the first week of each cycle followed by one infusion of 10 mg/kg every 4 weeks. The primary objective of the open-label extension study was to assess the long-term safety and tolerability of PRM-151, which were assessed by analysing adverse events (AEs) up to week 76 in all patients who received at least one dose of PRM-151 during the open-label extension study. Exploratory efficacy analyses were done by assessing changes from baseline in percentage of predicted FVC and 6-min walking distance, with descriptive statistics to week 76 and with random-intercept mixed models to week 52. This study is registered with ClinicalTrials.gov, number NCT02550873, and with EudraCT, number 2014-004782-24. Findings: Of 116 patients who completed the double-blind treatment period, 111 entered the open-label extension study (74 from the PRM-151 group and 37 from the placebo group). 84 (76%) of 111 patients received concomitant IPF therapy (pirfenidone n=55 or nintedanib n=29). AEs were consistent with long-term IPF sequelae. 31 (28%) patients had serious AEs. Those occurring in two or more patients were pneumonia (six [5%] of 111), IPF exacerbation (four [4%]), IPF progression (four [4%]), and chest pain (two [2%]). 21 (19%) patients had severe AEs, of which IPF exacerbation and IPF progression each occurred in two (2%) patients. Two (2%) patients experienced life-threatening AEs (one had pneumonia and one had small-cell lung cancer extensive stage). A persistent treatment effect was observed for PRM-151 in patients who continued treatment, with a decline in percentage of predicted FVC of −3·6% per year and in 6-min walking distance of −10·5 m per year at week 52. In patients who started PRM-151 during the open-label extension study, compared with the slopes for placebo, decline reduced for percentage of predicted FVC (from −8·7% per year in weeks 0–28 to −0·9% per year in weeks 28–52, p

Published
2019
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39. Psychometric properties and minimal important differences of SF-36 in Idiopathic Pulmonary Fibrosis

Author

Veronika Müller, Philippe Bonniaud, Bruno Crestani, Andreas Guenther, Sabine Witt, María Asunción Nieto Barbero, Ekaterina Krauss, Larissa Schwarzkopf, Werner Seeger, Walter Klepetko, Carlo Vancheri, Athol U. Wells, Rolf Holle, Alberto Pesci, Martina Vasakova, Reiner Leidl, Witt, S, Krauss, E, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Klepetko, W, Seeger, W, Crestani, B, Leidl, R, Holle, R, Schwarzkopf, L, and Guenther, A

Subjects
0301 basic medicine, Male, Quality of life, medicine.medical_specialty, SF-36, Psychometrics, Cross-sectional study, Health Status, Idiopathic Pulmonary Fibrosis, Patient-reported Outcome, Quality Of Life, Rare Diseases, Idiopathic pulmonary fibrosis, Patient-reported outcome, Rare diseases, 03 medical and health sciences, 0302 clinical medicine, Floor effect, Surveys and Questionnaires, medicine, Criterion validity, Humans, Longitudinal Studies, Aged, lcsh:RC705-779, Idiopathic pulmonary fibrosi, MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, business.industry, Research, Reproducibility of Results, lcsh:Diseases of the respiratory system, Middle Aged, humanities, 030104 developmental biology, Cross-Sectional Studies, 030228 respiratory system, Physical therapy, Population study, Female, business, Follow-Up Studies
Abstract

Background Idiopathic pulmonary fibrosis (IPF) is a rare disease with a median survival of 3–5 years after diagnosis with limited treatment options. The aim of this study is to assess the psychometric characteristics of the Short Form 36 Health Status Questionnaire (SF-36) in IPF and to provide disease specific minimally important differences (MID). Methods Data source was the European IPF Registry (eurIPFreg). The psychometric properties of the SF-36 version 2 were evaluated based on objective clinical measures as well as subjective perception. We analysed acceptance, feasibility, discrimination ability, construct and criterion validity, responsiveness and test-retest-reliability. MIDs were estimated via distribution and anchor-based approaches. Results The study population included 258 individuals (73.3% male; mean age 67.3 years, SD 10.7). Of them 75.2% (194 individuals) had no missing item. The distribution of several items was skewed, although floor effect was acceptable. Physical component score (PCS) correlated significantly and moderately with several anchors, whereas the correlations of mental component score (MCS) and anchors were only small. The tests showed mainly significant lower HRQL in individuals with long-term oxygen therapy. Analyses in stable individuals did not show significant changes of HRQL except for one dimension and anchor. Individuals with relevant changes of the health status based on the anchors had significant changes in all SF-36 dimensions and summary scales except for the dimension PAIN. PCS and MCS had mean MIDs of five and six, respectively. Mean MIDs of the dimensions ranged from seven to 21. Conclusion It seems that the SF-36 is a valid instrument to measure HRQL in IPF and so can be used in RCTs or individual monitoring of disease. Nevertheless, the additional evaluation of longitudinal aspects and MIDs can be recommended to further analyse these factors. Our findings have a great potential impact on the evaluation of IPF patients. Trial registration The eurIPFreg and eurIPFbank are listed in https://clinicaltrials.gov (NCT02951416). Electronic supplementary material The online version of this article (10.1186/s12931-019-1010-5) contains supplementary material, which is available to authorized users.

Published
2019

40. The European IPF registry (eurIPFreg): Baseline characteristics and survival of patients with idiopathic pulmonary fibrosis

Author

Günther, Andreas, Krauss, Ekaterina, Tello, Silke, Wagner, Jasmin, Paul, Bettina, Kuhn, Stefan, Maurer, Olga, Heinemann, Sabine, Costabel, Ulrich, Nieto Barbero, María Asunción, Müller, Veronika, Bonniaud, Philippe, Vancheri, Carlo, Wells, Athol, Vasakova, Martina, Pesci, Alberto, Sofia, Matteo, Klepetko, Walter, Seeger, Werner, Drakopanagiotakis, Fotios, Crestani, Bruno, Guenther, A, Krauss, E, Tello, S, Wagner, J, Paul, B, Kuhn, S, Maurer, O, Heinemann, S, Costabel, U, Barbero, M, Müller, V, Bonniaud, P, Vancheri, C, Wells, A, Vasakova, M, Pesci, A, Sofia, M, Klepetko, W, Seeger, W, Drakopanagiotakis, F, Crestani, B, European IPF Registry and Biobank (eurIPFreg/bank), University of Giessen and Marburg Lung Center (UGMLC), Justus-Liebig-Universität Gießen (JLU), Johann Wolfgang Goethe University, Goethe-Universität Frankfurt am Main, Agaplesion Pneumologische KliniK Waldhof-Elgershausen, German Center for Lung Research, University Hospital [Essen, Germany], Hospital Clinico San Carlos (Madrid), Semmelweis University [Budapest], Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale (INSERM), Università degli studi di Catania [Catania], Department of Respiratory Medicine, Royal Brompton Hospital, 1st Faculty of Medicine and Thomayer University Hospital, San Gerardo Hospital, Università degli studi di Napoli Federico II, University Hospital Vienna, Centre de compétences maladies pulmonaires rares [CHU Bichat], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and Medizinische Klinik II

Subjects
Male, Pulmonary and Respiratory Medicine, Idiopathic pulmonary fibrosis (IPF), Biopsy, European registry for idiopathic pulmonary fibrosis (eurIPFreg), Interstitial lung diseases (ILD), Aged, Aged, 80 and over, Cohort Studies, Europe, Female, Humans, Idiopathic Pulmonary Fibrosis, Longitudinal Studies, Lung, Survival Rate, Registries, Medizin, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 80 and over, ddc:610, lcsh:RC705-779, MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, Research, lcsh:Diseases of the respiratory system, respiratory system, Medical sciences Medicine, respiratory tract diseases
Abstract

Background: Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research.Methods: The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management.Results: This paper describes baseline data of 525 IPF subjects recruited from 11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value, DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under which patients showed improved survival (p = 0.001).Conclusions: Our data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time.Trial registration: The eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov(NCT02951416).

Published
2018
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41. Antacid therapy in idiopathic pulmonary fibrosis: more questions than answers?

Author

Claudia Valenzuela, Jan C. Grutters, David M. Hansell, Sebastiano Alfio Torrisi, Wim Wuyts, Nunzio Crimi, Maria Molina-Molina, Athol U. Wells, Benjamin Bondue, Andreas Guenther, Simon L.F. Walsh, Harold R. Collard, Raphael Borie, Thomas Geiser, Irina Strâmbu, Goksel Altinisik, Nathan Hambly, Martina Sterclova, Nesrin Mogulkoc, Marlies S. Wijsenbeek, Jack Gauldie, Martina Vasakova, Michael Kreuter, Sergio Harari, Elisabeth Bendstrup, Kevin K. Brown, Vincent Cottin, Venerino Poletti, Philippe Camus, Michael P. Keane, Luca Richeldi, Brett Ley, Claudia Ravaglia, Stefano Palmucci, Toby M. Maher, Eliza Tsitoura, Antje Prasse, Diego Castillo, Paola Rottoli, Carlo Albera, Kerri A. Johannson, Fabrizio Luppi, Aryeh Fischer, Paolo Spagnolo, Mark Jones, Carlo Vancheri, Katerina M. Antoniou, Giovanni Ferrara, Johannson, K, Strâmbu, I, Ravaglia, C, Grutters, J, Valenzuela, C, Mogulkoc, N, Luppi, F, Richeldi, L, Wells, A, Vancheri, C, Kreuter, M, Albera, C, Antoniou, K, Altinisik, G, Bendstrup, E, Bondue, B, Borie, R, Brown, K, Camus, P, Castillo, D, Collard, H, Cottin, V, Crimi, N, Ferrara, G, Fischer, A, Gauldie, J, Geiser, T, Guenther, A, Hambly, N, Hansell, D, Harari, S, Jones, M, Keane, M, Ley, B, Maher, T, Molina-Molina, M, Palmucci, S, Poletti, V, Prasse, A, Rottoli, P, Spagnolo, P, Sterclova, M, Torrisi, S, Tsitoura, E, Vasakova, M, Walsh, S, Wijsenbeek, M, and Wuyts, W

Subjects
Parenchymal lung disease, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Proton Pump Inhibitor, MEDLINE, Histamine H2 Antagonist, Settore MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, Severity of Illness Index, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Antacid therapy, Risk Factors, Antacids, Disease Progression, Gastroesophageal Reflux, Histamine H2 Antagonists, Humans, Idiopathic Pulmonary Fibrosis, Practice Guidelines as Topic, Proton Pump Inhibitors, Severity of illness, medicine, Antacid, 030212 general & internal medicine, Intensive care medicine, business.industry, Risk Factor, Disease progression, Idiopathic Pulmonary Fibrosi, Guideline, respiratory system, medicine.disease, respiratory tract diseases, 030228 respiratory system, Physical therapy, Position paper, business, Human
Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive parenchymal lung disease of complex cause. Gastro-oesophageal reflux (GER) and microaspiration have been proposed as risk factors for the development and progression of IPF, but robust definitive data are few. A recent international guideline conditionally recommended the use of antacid therapy (proton pump inhibitors or histamine-2-receptor antagonists) for patients with IPF, in the absence of oesophageal reflux or symptoms. In this Position Paper, we summarise the literature addressing the association between GER and IPF, and also identify future research priorities that could clarify this issue. We shed light on the process through which the guideline recommendation was achieved and aim to contextualise the recommendation for providers caring for patients with IPF.

Published
2017

42. Childhood interstitial lung disease survivors in adulthood: a European collaborative study.

Author

Manali ED, Griese M, Nathan N, Uzunhan Y, Borie R, Michel K, Schwerk N, Fijolek J, Radzikowska E, Chua F, Pabary R, Mogulkoc N, McCarthy C, Kallieri M, Papaioannou AI, Kiper N, Koziar Vasakova M, Lacina L, Molina-Molina M, Torrent-Vernetta A, Tsiligiannis T, Karadag B, Kokosi M, Renzoni EA, van Moorsel CHM, Campo I, Bendstrup E, Prior TS, Prasse A, Bonella F, Cottin V, Diesler R, Froidure A, Kolilekas L, Fotis L, Douros K, Kaditis AG, Jeny F, Chauveau S, Nunes H, Dahbia A, Mariani F, van der Vis JJ, Groen K, Erdem Eralp E, Gokdemir Y, Kocakaya D, Olgun Yildizeli S, Yalçın E, Emiralioğlu N, Nayir Buyuksahin H, O'Brien H, Karcıoglu O, Can D, Ezircan A, Kartal Ozturk G, Ocal N, Yuksel H, Narin Tongal S, Safrankova M, Kourtesi K, Louvrier C, Kannengiesser C, Fabre A, Legendre M, Crestani B, Pohunek P, Bush A, and Papiris SA

Subjects
Humans, Male, Female, Adolescent, Europe epidemiology, Child, Adult, Young Adult, Vital Capacity, Prognosis, Lung Diseases, Interstitial diagnosis, Lung Diseases, Interstitial physiopathology, Lung Diseases, Interstitial epidemiology, Survivors statistics & numerical data
Abstract

Background: Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood., Aim: To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres., Methods: This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected., Results: 244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients, respectively. Not all childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications., Conclusion: Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management., Competing Interests: Conflict of interest: E.D. Manali reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, CSL Behring and Hoffman-La Roche; support for attending meetings and/or travel from Boehringer Ingelheim, CSL Behring, Hoffman-La Roche and Elpen; and leadership as Chair in the ERS task force for transition of chILD. M. Griese reports consulting fees and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. N. Nathan reports grants from CORTICONEHI for the clinical trial “Efficacy of methylprednisolone pulses in neuroendocrine cells hyperplasia of infancy”, the Million Dollar Bike Ride project for Neuroendocrine Cell Hyperplasia of Infancy, the Chancellerie des Universités, RespiFIL for the development of an e-learning module for CT-scan in childhood interstitial lung diseases and RespiFIL for the development of an online platform for rare lung disease quality of life and transition questionnaires; payment or honoraria for lectures, presentations, manuscript writing or educational events from La Lettre du Pneumologue; and support for attending meetings from the ERS. Y. Uzunhan reports grants from Oxyvie; consulting fees from Boehringer Ingelheim and Pfizer; payment or honoraria for lectures, presentations, manuscript writing or educational events from Sanofi, CSL Vifor and Boehringer Ingelheim; support for attending meetings and/or travel from Boehringer Ingelheim and Oxyvie; and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. R. Borie reports consulting fees from Boehringer Ingelheim, Ferrer and Sanofi; payment or honoraria for lectures, presentations; and educational events from Boehringer Ingelheim; and support for attending meetings from Boehringer Ingelheim. N. Schwerk reports consulting fees from Boehringer Ingelheim for consultancy work; paid lectures, publications and advisory board participation; and a leadership role as President-elect of the German Society for Paediatric Pneumology. C. McCarthy reports support for the present manuscript from Savara Inc.; grants from Health Research Board (Ireland), Enterprise Ireland and The LAM Foundation; consultancy fees from Savara Inc., AI Therapeutics, and Theravance Inc.; support for attending meetings from Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with Savara Inc. M. Koziar Vasakova reports consulting fees from Boehringer Ingelheim and Roche; payment or honoraria for lectures, presentations and educational events from Boehringer Ingelheim and Roche; support for attending meetings from Roche and Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with MSD and Boehringer Ingelheim. M. Molina-Molina reports grants from Boehringer Ingelheim and Roche; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Ferrer and Veracyte. C.H.M. van Moorsel reports grants from Boehringer Ingelheim for research support in digital auscultation for ILD, payment or honoraria for lectures from Boehringer Ingelheim and a leadership role as Co-chair of the ClinGen ILD Gene Curation Expert Panel. I. Campo reports consulting fees from Partner Therapeutics for a paid interview and participation on a data safety monitoring board or advisory board with Savara as a member of the advisory board for molgramostim. E. Bendstrup reports payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Boehringer Ingelheim and Daichii Sankyo; support for attending meetings from Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with Simbec-Orion for Molecure DSMB. T.S. Prior reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim; and support for attending meetings from Boehringer Ingelheim. F. Bonella reports consulting fees from Boehringer Ingelheim, Sanofi, BMS and CSL-Behring; payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Sanofi; support for attending meetings from Boehringer Ingelheim, AstraZeneca and Atyr; and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Sanofi and BMS. R. Diesler reports grants from CSL Behring, Fondation du Souffle and Fondation pour la Recherche Médicale, and support for attending meetings from Asdia. A. Froidure reports consulting fees from Boehringer Ingelheim; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, AstraZeneca and GSK. F. Jeny reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim; and support for attending meetings from Oxyvie (travel for a French congress). N. Ocal reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Nobel and Humanis. B. Crestani reports grants from Boehringer Ingelheim; consultancy fees from BMS, Boehringer Ingelheim, Chiesi, GSK and Sanofi; payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, BMS, Boehringer Ingelheim, GSK, Novartis, Roche and Sanofi; support for attending meetings from AstraZeneca, BMS, Boehringer Ingelheim, Roche and Sanofi; participation on a data safety monitoring board or advisory board with BMS, Boehringer Ingelheim, Horizon and Sanofi; and a leadership role as President of the board of trustees of the Fondation du Souffle (a French charity). P. Pohunek reports support for the present study from the ERS Task Force TF2021-14 (payment for the librarian); consulting fees from AstraZeneca and GSK; payment or honoraria for lectures, presentations, manuscript writing or educational events from GSK and AstraZeneca (personal), and Chiesi, AstraZeneca and GSK (institutional); support for attending meetings from GSK and AstraZeneca; and participation on a data safety monitoring board or advisory board with GSK and AstraZeneca. S.A. Papiris reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Hoffman La Roche; support for attending meetings from Boehringer Ingelheim, Hoffman La Roche and Elpen; and leadership or fiduciary role as a member in the ERS Task Force for the transition of chILD. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2025. For reproduction rights and permissions contact permissions@ersnet.org.)

Published
2025
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44. Flow cytometry-based method using diversity of cytokine production differentiates between Mycobacterium tuberculosis infection and disease.

Author

Dolezalova K, Hadlova P, Ibrahimova M, Golias J, Baca L, Kopecka E, Sukholytka M, and Koziar Vasakova M

Subjects
Adolescent, Adult, Aged, Child, Child, Preschool, Female, Humans, Infant, Male, Middle Aged, Young Adult, Biomarkers analysis, Biomarkers metabolism, CD4-Positive T-Lymphocytes immunology, CD4-Positive T-Lymphocytes metabolism, CD8-Positive T-Lymphocytes immunology, CD8-Positive T-Lymphocytes metabolism, Diagnosis, Differential, Feasibility Studies, Interferon-gamma Release Tests statistics & numerical data, Mycobacterium tuberculosis immunology, Mycobacterium tuberculosis isolation & purification, Pilot Projects, Predictive Value of Tests, Prospective Studies, Tuberculin Test statistics & numerical data, Flow Cytometry methods, Flow Cytometry statistics & numerical data, Interferon-gamma analysis, Interferon-gamma immunology, Interferon-gamma metabolism, Interleukin-2 analysis, Interleukin-2 immunology, Interleukin-2 metabolism, Latent Tuberculosis blood, Latent Tuberculosis diagnosis, Latent Tuberculosis immunology, Latent Tuberculosis microbiology, Tuberculosis, Pulmonary blood, Tuberculosis, Pulmonary diagnosis, Tuberculosis, Pulmonary immunology, Tuberculosis, Pulmonary microbiology, Tumor Necrosis Factor-alpha analysis, Tumor Necrosis Factor-alpha immunology, Tumor Necrosis Factor-alpha metabolism
Abstract

Authors present a pilot study of the development of innovative flow cytometry-based assay with a potential for use in tuberculosis diagnostics. Currently available tests do not provide robust discrimination between latent tuberculosis infection (TBI) and tuberculosis disease (TB). The desired application is to distinguish between the two conditions by evaluating the production of a combination of three cytokines: IL-2 (interleukin-2), IFNɣ (interferon gamma) and TNFɑ (tumor necrosis factor alpha) in CD4 + and CD8 + T cells. The study was conducted on 68 participants, divided into two arms according to age (paediatric and adults). Each arm was further split into three categories (non-infection (NI), TBI, TB) based on the immune reaction to Mycobacterium tuberculosis (M.tb) after a close contact with pulmonary TB. Each blood sample was stimulated with specific M.tb antigens present in QuantiFERON tubes (TB1 and TB2). We inferred TBI or TB based on the predominant cytokine response of the CD4 + and/or CD8 + T cells. Significant differences were detected between the NI, TBI and the TB groups in TB1 in the CD4 + TNFɑ + parameter in children. Along with IL-2, TNFɑ seems to be the most promising diagnostic marker in both CD4 + and CD8 + T cells. However, more detailed analyses on larger cohorts are needed to confirm the observed tendencies., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. Signed: Karolina Dolezalova as the corresponding author on behalf of all authors listed., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Published
2024
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45. Adherence to the ISHLT Protocol for the Referral of Patients with Idiopathic Pulmonary Fibrosis to the Transplantation Center among of Czech Centers for Interstitial Lung Diseases.

Author

Sterclova M, Doubkova M, Sykorova L, Bartos V, Zurkova M, Lostakova V, Mokosova R, Plackova M, Lacina L, Cimrova M, Bittenglova R, Lisa P, Musilova P, Dolezal D, Psikalova J, Ovesna P, and Koziar Vasakova M

Subjects
Humans, Czech Republic, Middle Aged, Female, Male, Retrospective Studies, Guideline Adherence statistics & numerical data, Lung Diseases, Interstitial surgery, Registries, Adult, Aged, Idiopathic Pulmonary Fibrosis surgery, Lung Transplantation statistics & numerical data, Referral and Consultation statistics & numerical data
Abstract

There are limited data on referral rates and the number of patients with idiopathic pulmonary fibrosis (IPF) who are eligible for lung transplantation. The aim of the present study was to assess adherence to the consensus of the International Society for Heart and Lung Transplantation (ISHLT) for the referral of patients with IPF among Czech interstitial lung disease (ILD) centers. Czech patients who were diagnosed with IPF between 1999 and 2021 ( n = 1584) and who were less than 65 years old at the time of diagnosis were retrospectively selected from the Czech Republic of the European Multipartner Idiopathic Pulmonary Fibrosis Registry (EMPIRE). Nonsmokers and ex-smokers with a body mass index (BMI) of <32 kg/m 2 ( n = 404) were included for further analyses. Patients with a history of cancer <5 years from the time of IPF diagnosis, patients with alcohol abuse, and patients with an accumulation of vascular comorbidities were excluded. The trajectory of individual patients was verified at the relevant ILD center. From the database of transplant patients (1999-12/2021, n = 541), all patients who underwent transplantation for pulmonary fibrosis ( n = 186) were selected, and the diagnosis of IPF was subsequently verified from the patient's medical records ( n = 67). A total of 304 IPF patients were eligible for lung transplantation. Ninety-six patients were referred to the transplant center, 50% ( n = 49) of whom were referred for lung transplantation. Thirty percent of potentially eligible patients not referred to the transplant center were considered to have too many comorbidities by the reporting physician, 19% of IPF patients denied lung transplantation, and 17% were not referred due to age. Among Czech patients with IPF, there may be a larger pool of potential lung transplant candidates than has been reported to the transplant center to date., Competing Interests: The authors have no conflicts of interest, as we declare., (Copyright © 2024 Martina Sterclova et al.)

Published
2024
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46. CCL2, CCL8, CXCL12 chemokines in resectable non-small cell lung cancer (NSCLC).

Author

Drosslerova M, Sterclova M, Taskova A, Hytych V, Richterova E, Bruzova M, Spunda M, Komarc M, and Koziar Vasakova M

Subjects
Humans, Chemokines, Biomarkers, Chemokine CCL8, Chemokine CCL2, Chemokine CXCL12, Carcinoma, Non-Small-Cell Lung surgery, Carcinoma, Non-Small-Cell Lung pathology, Lung Neoplasms surgery, Lung Neoplasms pathology
Abstract

Background: Complex networks of chemokines are part of the immune reaction targeted against tumor cells. Chemokines influence cancer growth. It is unclear whether the concentrations of chemokines at the time of NSCLC (non-small cell lung cancer) diagnosis differ from healthy controls and reflect the extent of NSCLC., Aims: To compare chemokine concentrations (CCL2, CCL8, CXCL12) in the plasma of patients with resectable NSCLC to those without cancer. To determine whether the chemokine concentrations differ relative to the stage of disease., Methods: Sixty-nine patients undergoing surgery for proven/suspected NSCLC were enrolled. They underwent standard diagnostic and staging procedures to determine resectability, surgery was performed. Forty-two patients were diagnosed with NSCLC, while 27patients had benign lung lesions and functioned as the control group. Chemokine concentrations in peripheral blood were assessed using ELISA. Parametric statistics were used for the analysis of results., Results: There were no differences in plasma chemokine concentrations in NSCLC patients compared to controls. CXCL12 concentrations correlated positively with tumor extent expressed as clinical stage, (mean values: stage I 5.08 ng/mL, SEM 0.59; stage II and IIIA 7.82 ng/mL; SEM 1.06; P=0.022). Patients with NSCLC stages II+IIIA had significantly higher CXCL12 concentrations than controls (mean values: stage II+IIIA 7.82 ng/mL; SEM 1.06; controls 5.3 ng/mL; SEM 0.46; P=0.017)., Conclusion: CXCL12 was related to tumor growth and could potentially be used as a biomarker of advanced disease., Competing Interests: The authors report no conflicts of interest in this work.

Published
2023
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48. Long-term evaluation of the safety and efficacy of recombinant human pentraxin-2 (rhPTX-2) in patients with idiopathic pulmonary fibrosis (IPF): an open-label extension study.

Author

Raghu G, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Burgess T, Kamath N, Donaldson F, and Richeldi L

Subjects
Humans, Treatment Outcome, Vital Capacity, Idiopathic Pulmonary Fibrosis drug therapy, Recombinant Proteins adverse effects
Abstract

Background: Recombinant human pentraxin-2 (rhPTX-2) significantly decreased decline in percent predicted forced vital capacity (FVC) and stabilized 6-min walk distance (6MWD) in patients with idiopathic pulmonary fibrosis (IPF) during the 28-week, placebo-controlled, randomized period of the Phase II PRM-151-202 study. Interim (76-week) data from the open-label extension (OLE) demonstrated sustained safety and efficacy with rhPTX-2 treatment. Here, we present the entire long-term OLE safety and efficacy data to 128 weeks., Methods: Patients who completed the randomized PRM-151-202 study period were eligible for the OLE, during which all patients received rhPTX-2, having started rhPTX-2 (i.e., crossed from placebo) or continued rhPTX-2 after Week 28. rhPTX-2 was administered in 28-week cycles, with 10 mg/kg intravenous infusions (60 min) on Days 1, 3, and 5 in the first week of each cycle, then one infusion every 4 weeks up to Week 128. The OLE primary objective was to assess the long-term safety and tolerability of rhPTX-2. Other outcomes included FVC, 6MWD, and patient-reported outcomes (descriptive analysis)., Results: All 111 patients who completed the randomized period entered the OLE (n = 37 started rhPTX-2; n = 74 continued rhPTX-2); 57 (51.4%) completed to Week 128. The treatment-emergent adverse event (TEAE) profile was consistent with the randomized period, with the majority of TEAEs graded mild or moderate. Serious TEAEs occurred in 47 patients (42.3%), most frequently IPF (n = 11; 9.9%), pneumonia (n = 7; 6.3%), and acute respiratory failure (n = 3; 2.7%). Three patients underwent lung transplantation. Most serious TEAEs (and all 14 fatal events) were considered unrelated to rhPTX-2 treatment. For patients starting vs continuing rhPTX-2, mean (95% confidence interval) changes from baseline to Week 128 were, respectively, - 6.2% (- 7.7; - 4.6) and - 5.7% (- 8.0; - 3.3) for percent predicted FVC and - 36.3 m (- 65.8; - 6.9) and - 28.9 m (- 54.3; - 3.6) for 6MWD; however, conclusions were limited by patient numbers at Week 128., Conclusions: Long-term treatment (up to 128 weeks) with rhPTX-2 was well tolerated in patients with IPF, with no new safety signals emerging in the OLE. The limited efficacy data over 128 weeks may suggest a trend towards a treatment effect. Trial registration NCT02550873; EudraCT 2014-004782-24., (© 2022. The Author(s).)

Published
2022
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49. Lung Cancer in Non-smokers in Czech Republic: Data from LUCAS Lung Cancer Clinical Registry.

Author

Venclicek O, Skrickova J, Brat K, Fischer O, Havel L, Hrnciarik M, Marel M, Opalka P, Krakorova G, Rozsivalova D, Kultan J, Mullerova A, Zarnayova L, Smickova P, Vasakova M, Gyorfy Z, Jirousek M, Krejci D, Krejci J, Zuna P, Svaton M, Hrda K, Duba J, Alahakoon J, Svoboda M, and Silar J

Subjects
Adult, Aged, Aged, 80 and over, Biomarkers, Tumor genetics, Carcinoma, Non-Small-Cell Lung diagnosis, Carcinoma, Non-Small-Cell Lung mortality, Carcinoma, Non-Small-Cell Lung surgery, Czech Republic epidemiology, Ex-Smokers, Female, Humans, Lung Neoplasms diagnosis, Lung Neoplasms mortality, Lung Neoplasms surgery, Male, Middle Aged, Mutation, Neoplasm Staging, Pneumonectomy, Prospective Studies, Registries, Small Cell Lung Carcinoma diagnosis, Small Cell Lung Carcinoma mortality, Small Cell Lung Carcinoma therapy, Smokers, Time Factors, Treatment Outcome, Young Adult, Carcinoma, Non-Small-Cell Lung epidemiology, Lung Neoplasms epidemiology, Non-Smokers, Small Cell Lung Carcinoma epidemiology
Abstract

Background/aim: LUCAS is a clinical lung cancer registry (ClinicalTrials.gov identifier is NCT04228237), prospectively collecting data from newly diagnosed lung cancer patients in seven pneumooncology centers in the Czech Republic, since June 1, 2018. The aim of the study was to assess the stage of the disease at the time of diagnosis, percentage of morphological types, survival, percentage of driving mutations, eligibility for radical surgery, and percentage of patients who undergo radical surgery, in the non-smoking population in comparison with smokers and former smokers., Patients and Methods: The total number of patients in the registry at the time of the analysis was 2,743. Only 2,439 patients with complete records (smoking status, stage, and type of tumor) were included in this study., Results: The analysis indicated that non-smokers are diagnosed at a later stage of the disease but they have a better survival rate than smokers. Fewer smokers with stage III disease who are eligible for radical surgery will undergo surgery compared to non-smokers with the same clinical stage. Driving mutations are more common in non-smokers, even after adjustment for the more frequent occurrence of adenocarcinoma in the group of non-smokers., Conclusion: The data from LUCAS registry are consistent with already known facts, suggesting that the LUCAS registry is a useful clinical tool., (Copyright © 2021 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Published
2021
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50. Effect of genotype on the disease course in idiopathic pulmonary fibrosis despite antifibrotic treatment.

Author

Sterclova M, Kishore A, Sikorova K, Skibova J, Petrek M, and Vasakova M

Abstract

A genetic predisposition has been identified in 30% of idiopathic pulmonary fibrosis (IPF) cases. Although it is highly probable that the genotype affects the disease susceptibility and course in almost all patients, the specific genotype goes undetected. The aim of the present study was to explore the effects of variants of the genes encoding interleukin-4 (IL-4), mucin 5B (MUC5B), toll interacting protein (TOLLIP), surfactant protein A (SFPTA), transforming growth factor-β (TGF-β) and transporters associated with antigen processing (TAP1 and TAP2) on the course of IPF. A total of 50 patients with IPF were enrolled, and variants of these genes were assessed. Lung function at the time of diagnosis and after 6, 12 and 18 months, and the number of acute exacerbations and deaths in each observation period were measured. ANOVA was used to test the association between gene polymorphisms and the decrease in lung function. There was no significant effect of the gene polymorphisms on the outcomes of patients up to 6 months during the observation period. After 12 months, an effect of an IL-4 single nucleotide polymorphism (SNP) (rs 2070874) on patient outcomes was observed [relative risk (RR) for T allele: 5.6; 95% confidence interval (CI), 0.79-39.0; P=0.053]. The RR of progression in patients with the IL-4 SNP (rs 2243250) and the CT and TT genotypes was 4.3 (95% CI, 1.1-17.5; P=0.046). A total of 18 months after the diagnosis of IPF, an effect of the TOLLIP polymorphism on patient outcome was detected (rs 111521887; risk allele GC; RR: 7.2; 95% CI, 0.97-53.6; P=0.052). Thus, IL-4 and TOLLIP gene polymorphisms may represent disease course-modifying factors, but not drivers of IPF., Competing Interests: The authors declare that they have no competing interests., (Copyright: © Sterclova et al.)

Published
2021
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