Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: An international case-cohort study

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.; S. W., Ra; 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.

doi:10.1183/13993003.00936-2017

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 Cindex. 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, p<0.0001) than academic physicians (κw=0.56, IQR 0.45-0.65, p<0.0001) or physicians with access to multidisciplinary team (MDT) meetings (κw=0.54, IQR 0.45-0.64, p<0.0001). The prognostic accuracy of academic physicians with >20 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.