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1,371 results on '"Crestani, B."'

154. 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

155. Hot of the breath: Mortality as a primary end-point in IPF treatment trials: the best is the enemy of the good

Author

Wells, Athol U, Behr, Juergen, Costabel, Ulrich, Cottin, Vincent, Poletti, Venerino, Richeldi, Luca, Albera, C, Ancochea, J, Antoniou, KM, Bonella, F, Bonniaud, P, Bouros, D, Bresser, P, Cordier, JF, Crestani, B, Domagala-Kulawik, J, Drent, M, Egan, JJ, Geiser, T, Grunewald, J, Grutters, J, Gudmundsson, G, Guenther, A, Harari, S, Harrison, NK, Hirani, N, Hodgson, U, Kahler, CM, Keane, MP, Kiter, G, Kneussl, M, Maher, TM, Mogulkoc, N, Muller-Quernheim, J, Neurohr, C, Nunes, H, Parfrey, H, Peros-Golubicic, T, Polychronopoulos, V, Prevot, G, Renzoni, ER, Robalo Cordeiro, C, Saltini, C, Skold, M, Spagnolo, P, Thomeer, M, Tomasseti, S, Valeyre, D, Vancheri, C, Wallaert, B, Wuyts, W, and Xaubet, A

Published
2012
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161. Fibrose pulmonaire idiopathique au sein de la cohorte RaDiCo-PID

Author

Cottin, V., primary, Guéguen, S., additional, Jouneau, S., additional, Nunes, H., additional, Crestani, B., additional, Bonniaud, P., additional, Wemeau, L., additional, Israël-Biet, D., additional, Chevereau, M., additional, Dufaure-Garé, I., additional, Amselem, S., additional, and Clément, A., additional

Published
2020
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162. SAT0329 IS THE RATE OF LUNG FUNCTION DECLINE THE SAME IN PATIENTS WITH SYSTEMIC SCLEROSIS-ASSOCIATED ILD (SSC-ILD) WHO EXPERIENCE WEIGHT LOSS? DATA FROM THE SENSCIS TRIAL

Author

Lescoat, A., primary, Jouneau, S., additional, Crestani, B., additional, Riemekasten, G., additional, Kondoh, Y., additional, Smith, V., additional, Patel, N., additional, Huggins, J., additional, Stock, C., additional, Gahlemann, M., additional, Alves, M., additional, and Denton, C., additional

Published
2020
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163. OP0036 METHOTREXATE AND RHEUMATOID ARTHRITIS ASSOCIATED INTERSTITIAL LUNG DISEASE

Author

Juge, P. A., primary, Lee, J. S., additional, Lau, J., additional, Kawano, L., additional, Rojas-Serrano, J., additional, Sebastiani, M., additional, Koduri, G., additional, Matteson, E., additional, Bonfiglioli, K., additional, Sawamura, M., additional, Kairalla, R., additional, Cavagna, L., additional, Bozzalla Cassione, E., additional, Manfredi, A., additional, Mejia, M., additional, Rodríguez Henríquez, P., additional, Gonzalez-Perez, M. I., additional, Falfan-Valencia, R., additional, Buendia-Roldan, I., additional, Perez-Rubio, G., additional, Ebstein, E., additional, Gazal, S., additional, Borie, R., additional, Ottaviani, S., additional, Kannengiesser, C., additional, Wallaert, B., additional, Uzunhan, Y., additional, Nunes, H., additional, Valeyre, D., additional, Saidenberg Kermanac’h, N., additional, Boissier, M. C., additional, Wemeau Stervinou, L., additional, Flipo, R. M., additional, Marchand-Adam, S., additional, Richette, P., additional, Allanore, Y., additional, Dromer, C., additional, Truchetet, M. E., additional, Richez, C., additional, Schaeverbeke, T., additional, Lioté, H., additional, Thabut, G., additional, Deane, K., additional, Solomon, J., additional, Doyle, T., additional, Ryu, J. H., additional, Rosas, I. O., additional, Holers, V. M., additional, Boileau, C., additional, Debray, M. P., additional, Porcher, R., additional, Schwartz, D. A., additional, Vassallo, R., additional, Crestani, B., additional, and Dieudé, P., additional

Published
2020
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164. Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial

Author

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

Published
2020
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166. Clinical Outcomes at 1 Year in Patients with Idiopathic Pulmonary Fibrosis (IPF) Stratified by Annualized Weight Loss and Baseline Body Mass Index (BMI): A Post-Hoc Analysis from Ascend, Capacity, Inspire, and Riff

Author

Jouneau, S., primary, Crestani, B., additional, Thibault, R., additional, Lederlin, M., additional, Vernhet, L., additional, Yang, M., additional, Morgenthien, E., additional, Kirchgaessler, K.-U., additional, and Cottin, V., additional

Published
2020
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175. Performance diagnostique de la biopsie pulmonaire chirurgicale dans la prise en charge des pneumopathies interstitielles diffuses

Author

Le Guen, P., primary, Debray, M.P., additional, Mordant, P., additional, Guyard, A., additional, Iquille, J., additional, Taille, C., additional, Borie, R., additional, Dombret, M.C., additional, Ghanem, M., additional, Frija-Masson, J., additional, Bancal, C., additional, Khalil, A., additional, Cazes, A., additional, Castier, Y., additional, and Crestani, B., additional

Published
2020
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177. Incidence, caractéristiques et valeur pronostique de l’exacerbation subaiguë dans la fibrose pulmonaire idiopathique

Author

Caliez, J., primary, Sesé, L., additional, Cottin, V., additional, Brillet, P.Y., additional, Carton, Z., additional, Didier, M., additional, Israël-Biet, D., additional, Crestani, B., additional, Jouneau, S., additional, Cadranel, J., additional, Wallaert, B., additional, Tazi, A., additional, Maitre, 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, Valeyre, D., additional, and Nunes, H., additional

Published
2020
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178. Données démographiques et caractéristiques au moment du diagnostic des 847 patients atteints de fibrose pulmonaire idiopathique inclus dans la cohorte Radico-PID

Author

Cottin, V., primary, Jouneau, S., additional, Crestani, B., additional, Nunes, H., additional, Wemeau, L., additional, Bonniaud, P., additional, Israel-Biet, D., additional, Reynaud-Gaubert, M., additional, Dalphin, J.C., additional, Naccache, J.M., additional, Marchand-Adam, S., additional, Quetant, S., additional, Montani, D., additional, Hirschi, S., additional, Chevereau, M., additional, Dufaure-Garé, I., additional, and Clément, A., additional

Published
2020
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179. Pneumopathies interstitielles diffuses associées aux mutations de Poly(A)-specific ribonuclease (PARN) : une étude de cohorte rétrospective multicentrique

Author

Philippot, Q., primary, Kannengiesser, C., additional, Gondouin, A., additional, Naccache, J.M., additional, Bondue, B., additional, Israel Biet, D., additional, Mal, H., additional, Manali, E., additional, Papiris, S., additional, Wemeau, L., additional, Nunes, H., additional, Reynaud-Gaubert, M., additional, Schlemmer, F., additional, Cottin, V., additional, Crestani, B., additional, and Borie, R., additional

Published
2020
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180. Les inégalités de revenus influencent la survie sans progression dans la fibrose pulmonaire idiopathique

Author

Sesé, L., primary, Caliez, J., additional, Annesi-Maesano, I., additional, Cottin, V., additional, Didier, M., additional, Carton, Z., additional, Israël-Biet, D., additional, Crestani, B., additional, Jouneau, 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
2020
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181. Toll-like receptor 4 activation attenuates profibrotic response in control lung fibroblasts but not in fibroblasts from patients with IPF

Author

Ebener S, Barnowski S, Wotzkow C, Marti TM, Lopez-Rodriguez E, Crestani B, Blank F, Schmid RA, Geiser T, and Funke M

Subjects
respiratory system, respiratory tract diseases
Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease with a median survival of 3 yr. IPF deteriorates upon viral or bacterial lung infection although pulmonary infection (pneumonia) in healthy lungs rarely induces fibrosis. Bacterial lipopolysaccharide (LPS) activates Toll like receptor 4 (TLR4) initiating proinflammatory pathways. As TLR4 has already been linked to hepatic fibrosis and scleroderma we now investigated the role of TLR4 in IPF fibroblasts. Lung tissue sections from patients with IPF were analyzed for TLR4 expression. Isolated normal human lung fibroblasts (NL FB) and IPF fibroblasts (IPF FB) were exposed to LPS and transforming growth factor ß (TGF ß) before expression analysis of receptors profibrotic mediators and cytokines. TLR4 is expressed in fibroblast foci of IPF lungs as well as in primary NL FB and IPF FB. As a model for a gram negative pneumonia in the nonfibrotic lung NL FB and IPF FB were coexposed to LPS and TGF ß. Whereas NL FB produced significantly less connective tissue growth factor upon costimulation compared with TGF ß stimulation alone IPF FB showed significantly increased profibrotic markers compared with control fibroblasts after costimulation. Although levels of antifibrotic prostaglandin E2 were elevated after costimulation they were not responsible for this effect. However significant downregulation of TGF ß receptor type 1 in control fibroblasts seems to contribute to the reduced profibrotic response in our in vitro model. Normal and IPF fibroblasts thus differ in their profibrotic response upon LPS induced TLR4 stimulation.

Published
2017

183. Rheumatological evaluation of patients with interstitial lung disease.

Author

Ottaviani, S, Khaleche, S, Borie, R, Debray, M-P, Dieudé, P, and Crestani, B

Subjects
SYNOVITIS, INTERSTITIAL lung diseases, SJOGREN'S syndrome, CONNECTIVE tissue diseases, SYMPTOMS, PULMONARY function tests
Abstract

Objective: Interstitial lung disease (ILD) is a common feature of connective tissue disease (CTD). The diagnosis of CTD-ILD can be challenging and is important for therapeutic decisions. In this study, we aimed to determine whether a systematic rheumatological assessment could help pulmonologists in the diagnosis and care of ILD patients.Method: We conducted an observational single-centre study of patients with ILD. All patients underwent standardized pulmonary and rheumatological evaluations, including clinical evaluation (pulmonary symptoms and musculoskeletal signs), immunological screening, chest high-resolution computed tomography, pulmonary function tests, and ultrasonography (US) of joints and major salivary glands.Results: We included 100 consecutive ILD patients (47% women, mean ± sd age 67 ± 14 years); 15 patients already had CTD. The main extrapulmonary symptoms were joint pain (n = 52), joint swelling (n = 26), and sicca syndrome (n = 33). US of joints revealed synovitis, bone erosion, and tenosynovitis in 37, 17, and 13 patients, respectively. US of major salivary glands detected features associated with Sjögren's syndrome in 13 patients. After rheumatological evaluation, CTD-ILD was confidently diagnosed in 39 patients; diseases were mainly rheumatoid arthritis (n = 20), primary Sjögren's syndrome (n = 17), and inflammatory myopathies (n = 7). The diagnosis of CTD-ILD was associated with the presence of musculoskeletal symptoms and immunological and US abnormalities. The CTD diagnosis led to a therapeutic change in 21 patients.Conclusion: Our findings suggest that musculoskeletal symptoms are frequent in ILD patients, which supports multidisciplinary management, involving the rheumatologist, for evaluating patients with ILD. [ABSTRACT FROM AUTHOR]

Published
2022
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184. Excès de variants rares délétères au sein de gènes appartenant à la voir JAK-STAT chez des patients atteints de pneumopathie interstitielle diffuse associée à la polyarthrite rhumatoïde

Author

Juge, P.A., Gazal, S., Borie, R., Wemeau-Stervinou, L., Debray, M.P., Ottaviani, S., Marchand-Adam, S., Richez, C., Nunes, H., Richette, P., Kannengiesser, C., Froguel, P., Avouac, J., Sibilia, J., Flipo, R.M., Cottin, V., Schaeverbeke, T., Soubrier, M., Saidenberg-Kermanac’h, N., Dominique, V., Boileau, C., Crestani, B., and Dieudé, P.

Published
2022
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191. The characterisation of interstitial lung disease multidisciplinary team meetings: A global study

Author

Richeldi, L. Launders, N. Martinez, F. Walsh, S.L.F. Myers, J. Wang, B. Jones, M. Chisholm, A. Flaherty, K.R. David-Wang, A. Morais, A. Azuma, A. Crestani, B. Vancheri, C. Youakim, C. Fell, C.D. Ryerson, C.J. Bouros, D. Bendstrup, E. Morell, F. Bonella, F. Raghu, G. Christoff, G. Ferrara, G. Glaspole, I. Rosas, I. Behr, J. DeBoer, K. Antoniou, K.M. Dheda, K. Brown, K. Planas-Cerezales, L. Sköld, M. Funke, M. Molina-Molina, M. Mazzei, M. Kolb, M. Selman, M. Rottoli, P. Spagnolo, P. Rivera-Ortega, P. Avdeev, S. Quadrelli, S. Corte, T.J. Maher, T.M. Cottin, V. Wuyts, W. Xu, Z.J.

Subjects
body regions, respiratory system
Abstract

Multidisciplinary team (MDT) diagnosis of interstitial lung disease (ILD) has been proposed as a gold standard, but there are no formal recommendations for MDT process or composition and limited knowledge regarding prevalence in routine practice. We performed a systematic evaluation of ILD diagnostic practice across a range of healthcare settings around the world. Electronic questionnaires were distributed across all global regions via society and collaborators networks. Responses from 457 unique centres across 64 countries were included in the analysis. Of the 350 (76.6%) centres holding formal meetings, the majority held face-to-face MDT meetings (80%), for a minimum of 30 min (93%), and discussed diagnosis (96.9%) and patient management (94.9%) at the meetings. Compared with non-academic and academic non-ILD centres, ILD academic centres reported a higher ILD caseload, held more formal MDT meetings, and were more likely to include histopathology and rheumatology specialists in their diagnostic team. Of the centres holding MDT meetings, 5.5% routinely discussed all new cases at such meetings. An MDT approach to ILD diagnosis is consistently interpreted and widely implemented across a range of routine care settings around the world. This observation will inform future ILD diagnostic agreement studies and diagnostic pathway recommendations. © ERS 2019.

Published
2019

192. Myelodysplastic syndromes and idiopathic pulmonary fibrosis: A dangerous liaison

Author

Papiris, S.A. Tsirigotis, P. Kannengiesser, C. Kolilekas, L. Gkirkas, K. Papaioannou, A.I. Revy, P. Giouleka, P. Papadaki, G. Kagouridis, K. Pappa, V. Borie, R. Boileau, C. Bouros, D. Crestani, B. Manali, E.D.

Abstract

Previous studies have shown that the co-existence of bone marrow failure and pulmonary fibrosis in a single patient or in a family is suggestive of telomere related genes (TRG) germline mutations. This study presents the genetic background, clinical characteristics, and outcome of a group of five Greek patients co-affected with IPF and MDS. Four out of five patients developed an IPF acute exacerbation that was not reversible. We failed to detect any mutation in the TERT, TERC, DKC1, TINF2, RTEL1, PARN, NAF1, ACD, NHP2 and NOP10 genes in any patient. Moreover, telomere length was normal in the two patients tested. This could suggest that although the co-occurence of IPF and MDS are suggestive of TRG mutation in patients < 65 years old, in the elderly it may occur without germline mutations and could negatively affect prognosis. Physicians should be aware for possible IPF deterioration and therapeutic options for MDS should be wisely considered. © 2019 The Author(s).

Published
2019

196. Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis

Author

Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., Schwartz, D.A., Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., and Schwartz, D.A.

Abstract

Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods: We performed deep targeted resequencing (3.69 Mb of DNA) in cases (n = 3,624) and control subjects (n = 4,442) across genes and regions previously associated with disease. We tested for associations between disease and 1) individual common variants via logistic regression and 2) groups of rare variants via sequence kernel association tests. Measurements and Main Results: Statistically significant common variant association signals occurred in all 10 of the regions chosen based on genome-wide association studies. The strongest risk variant is the MUC5B promoter variant rs35705950, with an odds ratio of 5.45 (95% confidence interval, 4.91–6.06) for one copy of the risk allele and 18.68 (95% confidence interval, 13.34–26.17) for two copies of the risk allele (P = 9.60 × 10−295). In addition to identifying for the first time that rare variation in FAM13A is associated with disease, we confirmed the role of rare variation in the TERT and RTEL1 gene regions in the risk of IPF, and found that the FAM13A and TERT regions have independent common and rare variant signals. Conclusions: A limited number of common and rare variants contribute to the risk of idiopathic pulmonary fibrosis in each of the resequencing regions, and these genetic variants focus on biological mechanisms of host defense and cell senescence.

Published
2019

197. 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

198. Diagnostic likelihood thresholds that define a working diagnosis of idiopathic pulmonary fibrosis

Author

Walsh, S. L. F., Lederer, D. J., Ryerson, C. J., Kolb, M., Maher, T. M., Nusser, R., Poletti, V., Richeldi, Luca, Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K. K., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K. R., Glaspole, I. N., Grutters, J., Inoue, Y., Kondoh, Y., Kreuter, M., Johannson, K. A., Ley, B., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W. A., Wells, A. U., Richeldi L. (ORCID:0000-0001-8594-1448), Walsh, S. L. F., Lederer, D. J., Ryerson, C. J., Kolb, M., Maher, T. M., Nusser, R., Poletti, V., Richeldi, Luca, Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K. K., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K. R., Glaspole, I. N., Grutters, J., Inoue, Y., Kondoh, Y., Kreuter, M., Johannson, K. A., Ley, B., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W. A., Wells, A. U., and Richeldi L. (ORCID:0000-0001-8594-1448)

Abstract

Rationale: The level of diagnostic likelihood at which physicians prescribe antifibrotic therapy without requesting surgical lung biopsy (SLB) in patients suspected of idiopathic pulmonary fibrosis (IPF) is unknown. Objectives: To determine how often physicians advocate SLB in patient subgroups defined by IPF likelihood and risk associated with SLB, and to identify the level of diagnostic likelihood at which physicians prescribe antifibrotic therapy with requesting SLB. Methods: An international cohort of respiratory physicians evaluated 60 cases of interstitial lung disease, giving: 1) differential diagnoses with diagnostic likelihood; 2) a decision on the need for SLB; and 3) initial management. Diagnoses were stratified according to diagnostic likelihood bands described by Ryerson and colleagues. Measurements and Main Results: A total of 404 physicians evaluated the 60 cases (24,240 physician-patient evaluations). IPF was part of the differential diagnosis in 9,958/24,240 (41.1%) of all physician-patient evaluations. SLB was requested in 8.1%, 29.6%, and 48.4% of definite, provisional high-confidence and provisional low-confidence diagnoses of IPF, respectively. In 63.0% of provisional high-confidence IPF diagnoses, antifibrotic therapy was prescribed without requesting SLB. No significant mortality difference was observed between cases given a definite diagnosis of IPF (90-100% diagnostic likelihood) and cases given a provisional high-confidence IPF diagnosis (hazard ratio, 0.97; P = 0.65; 95% confidence interval, 0.90-1.04). Conclusions: Most respiratory physicians prescribe antifibrotic therapy without requesting an SLB if a provisional high-confidence diagnosis or "working diagnosis" of IPF can be made (likelihood>70%). SLB is recommended in only a minority of patients with suspected, but not definite, IPF.

Published
2019

199. The Ariane-IPF ERS Clinical Research Collaboration: seeking collaboration through launch of a federation of European registries on idiopathic pulmonary fibrosis

Author

Cottin, V., Annesi-Maesano, I., Gunther, A., Galvin, L., Kreuter, M., Powell, P., Prasse, A., Reynolds, G., Richeldi, L., Spagnolo, P., Valenzuela, C., Wijsenbeek, M., Wuyts, W. A., Crestani, B., Richeldi L. (ORCID:0000-0001-8594-1448), Cottin, V., Annesi-Maesano, I., Gunther, A., Galvin, L., Kreuter, M., Powell, P., Prasse, A., Reynolds, G., Richeldi, L., Spagnolo, P., Valenzuela, C., Wijsenbeek, M., Wuyts, W. A., Crestani, B., and Richeldi L. (ORCID:0000-0001-8594-1448)

Abstract

Rationale for a multinational registry Idiopathic pulmonary fibrosis (IPF) is the most prevalent of rare pulmonary diseases [1], and the most severe of the chronic forms of idiopathic interstitial pneumonias, with rising incidence and prevalence [2–4]. The prognosis for people diagnosed with IPF is a median survival of 3–3.5 years [1].

Published
2019

200. Safety and survival data in patients with idiopathic pulmonary fibrosis treated with nintedanib: Pooled data from six clinical trials

Author

Lancaster, L., Crestani, B., Hernandez, P., Inoue, Y., Wachtlin, D., Loaiza, L., Quaresma, M., Stowasser, S., Richeldi, Luca, Richeldi L. (ORCID:0000-0001-8594-1448), Lancaster, L., Crestani, B., Hernandez, P., Inoue, Y., Wachtlin, D., Loaiza, L., Quaresma, M., Stowasser, S., Richeldi, Luca, and Richeldi L. (ORCID:0000-0001-8594-1448)

Abstract

Introduction Nintedanib slows disease progression in patients with idiopathic pulmonary fibrosis (IPF) by reducing the rate of decline in forced vital capacity, with an adverse event profile that is manageable for most patients. We used data from six clinical trials to characterise the safety and tolerability profile of nintedanib and to investigate its effects on survival. Methods Data from patients treated with ≥1 dose of nintedanib 150 mg two times per day or placebo in the 52-week TOMORROW trial and/or its open-label extension; the two 52-week INPULSIS trials and/or their open-label extension, INPULSIS-ON; and a Phase IIIb trial with a placebo-controlled period of ≥6 months followed by open-label nintedanib were pooled. All adverse events, irrespective of causality, were included in descriptive analyses. Parametric survival distributions were fit to pooled Kaplan-Meier survival data from the trials and extrapolated to estimate long-term survival. Results There were 1126 patients in the pooled nintedanib group and 565 patients in the pooled placebo group. The mean duration of nintedanib treatment was 28 months. No new safety signals were observed. Incidence rates of bleeding, liver enzyme elevations and cardiovascular events were consistent with those observed in the INPULSIS trials. Diarrhoea was reported at a lower event rate in the pooled nintedanib group than in nintedanib-treated patients in the INPULSIS trials (76.5 vs 112.6 events per 100 patient exposure-years) and infrequently led to permanent treatment discontinuation (3.6 events per 100 patient exposure-years). Based on the Weibull distribution, mean (95% CI) survival was estimated as 11.6 (9.6, 14.1) years in nintedanib-treated patients and 3.7 (2.5, 5.4) years in placebo-treated patients. Conclusions Based on pooled data from six clinical trials, the adverse event profile of nintedanib was manageable for most patients. Exploratory analyses based on extrapolation of survival data suggest that nintedan

Published
2019

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