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579 results on '"Vancheri C."'

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

Author

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

Published
2020
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5. Chronic rhinosinusitis with nasal polyposis and biological agents: the ARIA-ITALY Survey

Author

Lombardi, C., primary, Passalacqua, G., additional, Menzella, F., additional, Mauritz Canevari, R.F, additional, Danesi, G., additional, Pusateri, A.M., additional, Carone, M., additional, Vancheri, C., additional, Di Marco, F., additional, Micheletto, C., additional, Manzotti, G., additional, Di Gioacchino, M, additional, Bilò, M.B, additional, Gelardi, M, additional, Senna, G, additional, Canonica, G.W, additional, and Italy Panel, ARIA, additional

Published
2024
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6. Diagnostic delay in bronchiectasis: an Italian perspective

Author

Chessarí, C, Simonetta, E, Amati, F, Nigro, M, Stainer, A, Sotgiu, G, Puci, M, Gramegna, A, Blasi, F, Morlacchi, L, Buscemi, A, Conio, V, Sanci, V, Corsico, A, Faverio, P, Michalak, W, Luppi, F, Crimi, C, Vancheri, C, Campisi, R, Vulpi, M, Carpagnano, G, Cicchetti, M, Sekretna, K, Scichilone, N, Battaglia, S, Aliberti, S, Morlacchi, LC, Buscemi, AAMD, Corsico, AG, Vulpi, MR, Carpagnano, GE, Chessarí, C, Simonetta, E, Amati, F, Nigro, M, Stainer, A, Sotgiu, G, Puci, M, Gramegna, A, Blasi, F, Morlacchi, L, Buscemi, A, Conio, V, Sanci, V, Corsico, A, Faverio, P, Michalak, W, Luppi, F, Crimi, C, Vancheri, C, Campisi, R, Vulpi, M, Carpagnano, G, Cicchetti, M, Sekretna, K, Scichilone, N, Battaglia, S, Aliberti, S, Morlacchi, LC, Buscemi, AAMD, Corsico, AG, Vulpi, MR, and Carpagnano, GE

Abstract

It takes ∼3.5 years to reach a diagnosis of bronchiectasis from onset of symptoms: the long patient's journey in Italy https://bit.ly/46XMWAz.

Published
2024

7. EPH200 Healthcare Resource Consumption of Patients with Chronic Obstructive Pulmonary Disease Treated with Triple and Dual Therapy and Experiencing an Acute Exacerbation

Author

Calabria, S., primary, Ronconi, G., additional, Dondi, L., additional, Piccinni, C., additional, Dell'Anno, I., additional, Pedrini, A., additional, Esposito, I., additional, Brignoli, O., additional, Canonica, G.W., additional, Carone, M., additional, Di Marco, F., additional, Micheletto, C., additional, Vancheri, C., additional, and Martini, N., additional

Published
2023
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8. Utility of ultrasound assessment of diaphragmatic function before and after pulmonary rehabilitation in COPD patients

Author

Crimi C, Heffler E, Augelletti T, Campisi R, Noto A, Vancheri C, and Crimi N

Subjects
diaphragm ultrasound, COPD, pulmonary rehabilitation, Diseases of the respiratory system, RC705-779
Abstract

Claudia Crimi,1 Enrico Heffler,2 Teresa Augelletti,2 Raffaele Campisi,1 Alberto Noto,3 Carlo Vancheri,4 Nunzio Crimi2 1Respiratory Medicine Unit, AOU “Policlinico-Vittorio Emanuele”, Catania, Italy; 2Respiratory Medicine Unit, Department of Clinical and Experimental Medicine, AOU “Policlinico-Vittorio Emanuele”, University of Catania, Catania, Italy; 3Anesthesia and Intensive Care Unit, AOU Policinico “G. Martino”, Messina, Italy; 4Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele”, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy Background: Pulmonary rehabilitation (PR) may improve respiratory symptoms and skeletal muscle strength in patients with COPD. We aimed to evaluate changes in ultrasound (US) measurements of diaphragmatic mobility and thickness after PR in COPD patients and to test its correlation with PR outcomes.Methods: Twenty-five COPD patients were enrolled and underwent a diaphragm US assessment before and after a 12-week PR program.Results: We found a correlation between the intraindividual percentage of change in the diaphragmatic length of zone of apposition at functional residual capacity (ΔLzapp%) and the change in 6-minute walking distance (6MWD) after PR (rho=0.49, P=0.02). ΔLzapp% was significantly higher in patients with improved 6MWD and COPD Assessment Test (CAT) score (mean rank=12.03±2.57 vs 6.88±4.37; P=0.02). A ΔLzapp% of ≥10% was able to discriminate among patients with improved 6MWD, with a sensitivity of 83% and a specificity of 74%. The area under the receiver operating characteristic curve for ΔLzapp% was 0.83. A cutoff value of ≥9% of ΔLzapp% had a positive predictive value in discriminating a reduction in ≥2 points of CAT score after PR, with a sensitivity and a specificity of 80% and 62%, respectively.Conclusion: Diaphragm US assessment represents a useful prognostic marker of PR outcomes in COPD patients. Keywords: diaphragm ultrasound, COPD, pulmonary rehabilitation

Published
2018

10. Hsa-miR223-3p circulating level is upregulated in Friedreich's ataxia and inversely associated with HCLS1 associated protein X-1, HAX-1

Author

Quatrana, A, Morini, E, Tiano, F, Vancheri, C, Panarello, L, Romano, S, Marcotulli, C, Casali, C, Mariotti, C, Mongelli, A, Fichera, M, Rufini, A, Condo, I, Novelli, G, Testi, R, Amati, F, Malisan, F, Quatrana A., Morini E., Tiano F., Vancheri C., Panarello L., Romano S., Marcotulli C., Casali C., Mariotti C., Mongelli A., Fichera M., Rufini A., Condo I., Novelli G., Testi R., Amati F., Malisan F., Quatrana, A, Morini, E, Tiano, F, Vancheri, C, Panarello, L, Romano, S, Marcotulli, C, Casali, C, Mariotti, C, Mongelli, A, Fichera, M, Rufini, A, Condo, I, Novelli, G, Testi, R, Amati, F, Malisan, F, Quatrana A., Morini E., Tiano F., Vancheri C., Panarello L., Romano S., Marcotulli C., Casali C., Mariotti C., Mongelli A., Fichera M., Rufini A., Condo I., Novelli G., Testi R., Amati F., and Malisan F.

Abstract

Frataxin (FXN) deficiency is responsible for Friedreich's ataxia (FRDA) in which, besides the characteristic features of spinocerebellar ataxia, two thirds of patients develop hypertrophic cardiomyopathy that often progresses to heart failure and premature death. Different mechanisms might underlie FRDA pathogenesis. Among them, the role of miRNAs deserves investigations. We carried out an miRNA PCR-array analysis of plasma samples of early-, intermediate- and late-onset FRDA groups, defining a set of 30 differentially expressed miRNAs. Hsa-miR223-3p is the only miRNA shared between the three patient groups and appears upregulated in all of them. The up-regulation of hsa-miR223-3p was further validated in all enrolled patients (n = 37, Fc = +2.3; P < 0.0001). Using a receiver operating characteristic curve analysis, we quantified the predictive value of circulating hsa-miR223-3p for FRDA, obtaining an area under the ROC curve value of 0.835 (P < 0.0001) for all patients. Interestingly, we found a significant positive correlation between hsa-miR223-3p expression and cardiac parameters in typical FRDA patients (onset < 25 years). Moreover, a significant negative correlation between hsa-miR223-3p expression and HAX-1 (HCLS1-associated protein X-1) at mRNA and protein level was observed in all FRDA patients. In silico analyses suggested HAX-1 as a target gene of hsa-miR223-3p. Accordingly, we report that HAX-1 is negatively regulated by hsa-miR223-3p in cardiomyocytes (AC16) and neurons (SH-SY5Y), which are critically affected cell types in FRDA. This study describes for the first time the association between hsa-miR223-3p and HAX-1 expression in FRDA, thus supporting a potential role of this microRNA as non-invasive epigenetic biomarker for FRDA.

Published
2022

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

Author

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

Abstract

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

Published
2022

13. Ziritaxestat, a novel autotaxin inhibitor, and lung function in idiopathic pulmonary fibrosis

Author

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

Abstract

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

Published
2023

15. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

Author

Kalluri, M., Luppi, F., Vancheri, A., Vancheri, C., Balestro, E., Varone, F., Mogulkoc, N., Cacopardo, G., Bargagli, E., Renzoni, E., Torrisi, S., Calvello, M., Libra, A., Pavone, M., Bonella, F., Cottin, V., Valenzuela, C., Wijsenbeek, M., Bendstrup, E., 3rd International Summit for ILD (ISILD), Erice collaborators listed below: Carlo Albera, Goksel, Altinisik, Kjetil, Ask, Elisabetta, Balestro, Elena, Bargagli, Elisabeth, Bendstrup, Marialuisa, Bocchino, Francesco, Bonella, Martina, Bonifazi, Giulia, Cacopardo, Maria, Calvello, Diego, M Castillo, Nazia, Chaudhuri, Ulrich, Costabel, Vincent, Cottin, Bruno, Crestani, Manuela, Funke-Chambour, Jack, Gauldie, Peter, M George, Johannes, C Grutters, Sergio, Harari, Richard, G Jenkins, Kerri, A Johannson, Mark, G Jones, Meena, Kalluri, Michael, P Keane, Maria, A Kokosi, Michael, Kreuter, Donato, Lacedonia, Brett, Ley, Alessandro, Libra, Fabrizio, Luppi, Toby, M Maher, George, A Margaritopoulos, Fernando, J Martinez, Jelle, Miedema, Nesrin, Mogulkoc, Maria, Molina-Molina, Philip, L Molyneaux, Julie, Morisset, Stefano, Palmucci, Mauro, Pavone, Ganesh, R Raghu, Elisabetta, A Renzoni, Luca, Richeldi, Gianluca, Sambataro, Alfredo, Sebastiani, Paolo, Spagnolo, Giulia Maria Stella, Martina, Sterclova, Irina, Strambu, Tomassetti, Sara, Sebastiano, Torrisi, Jacopo, Simonetti, Haluk, Turktas, Argyrios, Tzouvelekis, Claudia, Valenzuela, Ada, Vancheri, Carlo, Vancheri, Francesco, Varone, Patrizio, Vitulo, Athol, U Wells, Marlies, S Wijsenbeek, Wim, A Wuyts, Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, M., Luppi, F., Vancheri, A., Vancheri, C., Balestro, E., Varone, F., Mogulkoc, N., Cacopardo, G., Bargagli, E., Renzoni, E., Torrisi, S., Calvello, M., Libra, A., Pavone, M., Bonella, F., Cottin, V., Valenzuela, C., Wijsenbeek, M., Bendstrup, E., and Bocchino, M.

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Concordance, Health Status, interstitial lung dieseases, MEDLINE, Medizin, Prom, 03 medical and health sciences, 0302 clinical medicine, Medicine, Humans, 030212 general & internal medicine, Patient Reported Outcome Measures, Intensive care medicine, business.industry, Minimal clinically important difference, Interstitial lung disease, respiratory system, medicine.disease, Idiopathic Pulmonary Fibrosis, female genital diseases and pregnancy complications, respiratory tract diseases, Clinical trial, patient reported outcomes, patient reported outcome measures, idiopathic pulmonary fibrosi, 030228 respiratory system, Patient-reported outcome, Computerized adaptive testing, Patient Participation, business, Lung Diseases, Interstitial
Abstract

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Published
2021
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17. Clinical course of IPF in Italian patients during 12 months of observation: results from the FIBRONET observational study

Author

Poletti, V, Vancheri, C, Albera, C, Harari, S, Pesci, A, Metella, R, Campolo, B, Crespi, G, Rizzoli, S, Tomassetti, S, Rottoli, P, Bocchino, M, Stanziola, A, Luppi, F, Sebastiani, A, Lacedonia, D, Vitulo, P, Tavanti, L, Vianello, A, Saetta, M, Marinari, S, Pirina, P, Valente, S, Oggionni, T, Gasparini, S, Poletti V., Vancheri C., Albera C., Harari S., Pesci A., Metella R. R., Campolo B., Crespi G., Rizzoli S., Tomassetti S., Rottoli P., Bocchino M., Stanziola A. A., Luppi F., Sebastiani A., Lacedonia D., Vitulo P., Tavanti L., Vianello A., Saetta M., Marinari S., Pirina P., Valente S., Oggionni T., Gasparini S., Poletti, V, Vancheri, C, Albera, C, Harari, S, Pesci, A, Metella, R, Campolo, B, Crespi, G, Rizzoli, S, Tomassetti, S, Rottoli, P, Bocchino, M, Stanziola, A, Luppi, F, Sebastiani, A, Lacedonia, D, Vitulo, P, Tavanti, L, Vianello, A, Saetta, M, Marinari, S, Pirina, P, Valente, S, Oggionni, T, Gasparini, S, Poletti V., Vancheri C., Albera C., Harari S., Pesci A., Metella R. R., Campolo B., Crespi G., Rizzoli S., Tomassetti S., Rottoli P., Bocchino M., Stanziola A. A., Luppi F., Sebastiani A., Lacedonia D., Vitulo P., Tavanti L., Vianello A., Saetta M., Marinari S., Pirina P., Valente S., Oggionni T., and Gasparini S.

Abstract

Background: FIBRONET was an observational, multicentre, prospective cohort study investigating the baseline characteristics, clinical course of disease and use of antifibrotic treatment in Italian patients with idiopathic pulmonary fibrosis (IPF). Methods: Patients aged ≥ 40 years diagnosed with IPF within the previous 3 months at 20 Italian centres were consecutively enrolled and followed up for 12 months, with evaluations at 3, 6, 9 and 12 months. The primary objective was to describe the clinical course of IPF over 12 months of follow-up, including changes in lung function measured by % predicted forced vital capacity (FVC% predicted). Results: 209 patients (82.3% male, mean age 69.54 ± 7.43 years) were enrolled. Mean FVC% predicted was relatively preserved at baseline (80.01%). The mean time between IPF diagnosis and initiation of antifibrotic therapy was 6.38 weeks; 72.3% of patients received antifibrotic therapy within the first 3 months of follow-up, and 83.9% within 12 months of follow-up. Mean FVC% predicted was 80.0% at baseline and 82.2% at 12 months, and 47.4% of patients remained stable (i.e. had no disease progression) in terms of FVC% predicted during the study. Conclusions: FIBRONET is the first prospective, real-life, observational study of patients with IPF in Italy. The short time between diagnosis and initiation of antifibrotic therapy, and the stable lung function between baseline and 12 months, suggest that early diagnosis and prompt initiation of antifibrotic therapy may preserve lung function in patients with IPF. Trial registration: NCT02803580

Published
2021

18. Clinical course of IPF in Italian patients during 12 months of observation: results from the FIBRONET observational study

Author

Poletti V., Vancheri C., Albera C., Harari S., Pesci A., Metella R. R., Campolo B., Crespi G., Rizzoli S., Tomassetti S., Rottoli P., Bocchino M., Stanziola A. A., Luppi F., Sebastiani A., Lacedonia D., Vitulo P., Tavanti L., Vianello A., Saetta M., Marinari S., Pirina P., Valente S., Oggionni T., Gasparini S., Poletti, V, Vancheri, C, Albera, C, Harari, S, Pesci, A, Metella, R, Campolo, B, Crespi, G, Rizzoli, S, Tomassetti, S, Rottoli, P, Bocchino, M, Stanziola, A, Luppi, F, Sebastiani, A, Lacedonia, D, Vitulo, P, Tavanti, L, Vianello, A, Saetta, M, Marinari, S, Pirina, P, Valente, S, Oggionni, T, Gasparini, S, Poletti, V., Vancheri, C., Albera, C., Harari, S., Pesci, A., Metella, R. R., Campolo, B., Crespi, G., Rizzoli, S., Tomassetti, S., Rottoli, P., Bocchino, M., Stanziola, A. A., Luppi, F., Sebastiani, A., Lacedonia, D., Vitulo, P., Tavanti, L., Vianello, A., Saetta, M., Marinari, S., Pirina, P., Valente, S., Oggionni, T., and Gasparini, S.

Subjects
Male, Vital capacity, medicine.medical_specialty, Time Factors, Nintedanib, Vital Capacity, Idiopathic pulmonary fibrosis, Disease, Pirfenidone, 03 medical and health sciences, chemistry.chemical_compound, FEV1/FVC ratio, 0302 clinical medicine, Internal medicine, medicine, Humans, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, Observational, Aged, lcsh:RC705-779, Idiopathic pulmonary fibrosi, business.industry, Research, Anti-Inflammatory Agents, Non-Steroidal, Antifibrotic therapy, lcsh:Diseases of the respiratory system, respiratory system, medicine.disease, Prognosis, Lung function, respiratory tract diseases, 030228 respiratory system, chemistry, Italy, Real-world, Disease Progression, Observational study, Female, business, medicine.drug, Follow-Up Studies
Abstract

Background FIBRONET was an observational, multicentre, prospective cohort study investigating the baseline characteristics, clinical course of disease and use of antifibrotic treatment in Italian patients with idiopathic pulmonary fibrosis (IPF). Methods Patients aged ≥ 40 years diagnosed with IPF within the previous 3 months at 20 Italian centres were consecutively enrolled and followed up for 12 months, with evaluations at 3, 6, 9 and 12 months. The primary objective was to describe the clinical course of IPF over 12 months of follow-up, including changes in lung function measured by % predicted forced vital capacity (FVC% predicted). Results 209 patients (82.3% male, mean age 69.54 ± 7.43 years) were enrolled. Mean FVC% predicted was relatively preserved at baseline (80.01%). The mean time between IPF diagnosis and initiation of antifibrotic therapy was 6.38 weeks; 72.3% of patients received antifibrotic therapy within the first 3 months of follow-up, and 83.9% within 12 months of follow-up. Mean FVC% predicted was 80.0% at baseline and 82.2% at 12 months, and 47.4% of patients remained stable (i.e. had no disease progression) in terms of FVC% predicted during the study. Conclusions FIBRONET is the first prospective, real-life, observational study of patients with IPF in Italy. The short time between diagnosis and initiation of antifibrotic therapy, and the stable lung function between baseline and 12 months, suggest that early diagnosis and prompt initiation of antifibrotic therapy may preserve lung function in patients with IPF. Trial registration: NCT02803580

Published
2021
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19. The role of chest CT in deciphering interstitial lung involvement: systemic sclerosis versus COVID-19

Author

Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, Martina, Landini, Nicholas, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, Matucci-Cerinic, Marco, Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, Martina, Landini, Nicholas, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, and Matucci-Cerinic, Marco

Abstract

Objective: The aim of this study was to identify the main CT features that may help in distinguishing a progression of interstitial lung disease (ILD) secondary to SSc from COVID-19 pneumonia. Methods: This multicentric study included 22 international readers grouped into a radiologist group (RADs) and a non-radiologist group (nRADs). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Results: Fibrosis inside focal ground-glass opacities (GGOs) in the upper lobes; fibrosis in the lower lobe GGOs; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONs in the lower lobes (P < 0.0001) and signs of fibrosis in GGOs in the lower lobes (P < 0.0001) remained independently associated with COVID-19 pneumonia and SSc-ILD, respectively. A predictive score was created that was positively associated with COVID-19 diagnosis (96.1% sensitivity and 83.3% specificity). Conclusion: CT diagnosis differentiating between COVID-19 pneumonia and SSc-ILD is possible through a combination of the proposed score and radiologic expertise. The presence of consolidation in the lower lobes may suggest COVID-19 pneumonia, while the presence of fibrosis inside GGOs may indicate SSc-ILD.

Published
2022

24. Frataxin deficiency in Friedreich's ataxia is associated with reduced levels of HAX-1, a regulator of cardiomyocyte death and survival

Author

Tiano, F, Amati, F, Cherubini, F, Morini, E, Vancheri, C, Maletta, S, Fortuni, S, Serio, D, Quatrana, A, Luffarelli, R, Benini, M, Alfedi, G, Panarello, L, Rufini, A, Toschi, N, Frontali, M, Romano, S, Marcotulli, C, Casali, C, Gioiosa, S, Mariotti, C, Mongelli, A, Fichera, M, Condo, I, Novelli, G, Testi, R, Malisan, F, Tiano F., Amati F., Cherubini F., Morini E., Vancheri C., Maletta S., Fortuni S., Serio D., Quatrana A., Luffarelli R., Benini M., Alfedi G., Panarello L., Rufini A., Toschi N., Frontali M., Romano S., Marcotulli C., Casali C., Gioiosa S., Mariotti C., Mongelli A., Fichera M., Condo I., Novelli G., Testi R., Malisan F., Tiano, F, Amati, F, Cherubini, F, Morini, E, Vancheri, C, Maletta, S, Fortuni, S, Serio, D, Quatrana, A, Luffarelli, R, Benini, M, Alfedi, G, Panarello, L, Rufini, A, Toschi, N, Frontali, M, Romano, S, Marcotulli, C, Casali, C, Gioiosa, S, Mariotti, C, Mongelli, A, Fichera, M, Condo, I, Novelli, G, Testi, R, Malisan, F, Tiano F., Amati F., Cherubini F., Morini E., Vancheri C., Maletta S., Fortuni S., Serio D., Quatrana A., Luffarelli R., Benini M., Alfedi G., Panarello L., Rufini A., Toschi N., Frontali M., Romano S., Marcotulli C., Casali C., Gioiosa S., Mariotti C., Mongelli A., Fichera M., Condo I., Novelli G., Testi R., and Malisan F.

Abstract

Frataxin deficiency, responsible for Friedreich's ataxia (FRDA), is crucial for cell survival since it critically affects viability of neurons, pancreatic beta cells and cardiomyocytes. In FRDA, the heart is frequently affected with typical manifestation of hypertrophic cardiomyopathy, which can progress to heart failure and cause premature death. A microarray analysis performed on FRDA patient's lymphoblastoid cells stably reconstituted with frataxin, indicated HS-1-associated protein X-1 (HAX-1) as the most significantly upregulated transcript (FC = +2, P < 0.0006). quantitative Reverse Transcription-Polymerase Chain Reaction (qRT-PCR) and western blot analysis performed on (I) HEK293 stably transfected with empty vector compared to wild-type frataxin and (II) lymphoblasts from FRDA patients show that low frataxin mRNA and protein expression correspond to reduced levels of HAX-1. Frataxin overexpression and silencing were also performed in the AC16 human cardiomyocyte cell line. HAX-1 protein levels are indeed regulated through frataxin modulation. Moreover, correlation between frataxin and HAX-1 was further evaluated in peripheral blood mononuclear cells (PBMCs) from FRDA patients and from non-related healthy controls. A regression model for frataxin which included HAX-1, group membership and group∗ HAX-1 interaction revealed that frataxin and HAX-1 are associated both at mRNA and protein levels. Additionally, a linked expression of FXN, HAX-1 and antioxidant defence proteins MnSOD and Nrf2 was observed both in PBMCs and AC16 cardiomyocytes. Our results suggest that HAX-1 could be considered as a potential biomarker of cardiac disease in FRDA and the evaluation of its expression might provide insights into its pathogenesis as well as improving risk stratification strategies.

Published
2020

25. Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study

Author

Sgalla, G, Lo Greco, E, Calvello, M, Varone, F, Iovene, B, Cerri, S, Donatelli, P, Vancheri, A, Pavone, M, Luppi, F, Vancheri, C, Richeldi, L, Sgalla G., Lo Greco E., Calvello M., Varone F., Iovene B., Cerri S., Donatelli P., Vancheri A., Pavone M., Luppi F., Vancheri C., Richeldi L., Sgalla, G, Lo Greco, E, Calvello, M, Varone, F, Iovene, B, Cerri, S, Donatelli, P, Vancheri, A, Pavone, M, Luppi, F, Vancheri, C, Richeldi, L, Sgalla G., Lo Greco E., Calvello M., Varone F., Iovene B., Cerri S., Donatelli P., Vancheri A., Pavone M., Luppi F., Vancheri C., and Richeldi L.

Abstract

Background and objective: In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods: Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results: A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49–2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83–2.44, P = 0.201 for disease progression). Conclusion: In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

Published
2020

27. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: Where to go from here?

Author

Kalluri M., Luppi F., Vancheri A., Vancheri C., Balestro E., Varone F., and Mogulkoc N.

Subjects
interstitial lung disease, patient satisfaction, Health Status, minimal clinically important difference, cross validation, visual analog scale, phase 1 clinical trial (topic), methodology, Review, patient-reported outcome, Idiopathic Pulmonary Fibrosis, human experiment, fibrosing alveolitis, phase 4 clinical trial (topic), St. George Respiratory Questionnaire, validation process, phase 3 clinical trial (topic), phase 2 clinical trial (topic), Humans, human, Patient Reported Outcome Measures, Patient Participation, randomized controlled trial (topic), Lung Diseases, Interstitial, outcome assessment
Abstract

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth. © The authors 2021., AstraZeneca; Novartis; Sanofi; Actelion Pharmaceuticals; F. Hoffmann-La Roche; Bristol-Myers Squibb Canada, BMS; Boehringer Ingelheim; Shionogi, Conflict of interest: M. Kalluri reports grants and personal fees from Boehringer Ingelheim, and Roche, outside the submitted work. F. Luppi reports grants and lecture fees from Roche and lecture fees from Boehringer Ingelheim. A. Vancheri has nothing to disclose. C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work. E. Balestro reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. F. Varone reports unrestricted grants, consultancy and lecture fees from Roche and Boehringer Ingelheim. N. Mogulkoc has nothing to disclose. G. Cacopardo has nothing to disclose. E. Bargagli has nothing to disclose. E. Renzoni reports grants from Boeringher Ingelheim, and lecture fees from Boeringher Ingelheim and Roche, outside the submitted work. S. Torrisi reports personal fees from Hoffman La-Roche and Boehringer Ingelheim, outside the submitted work. M. Calvello has nothing to disclose. A. Libra has nothing to disclose. M. Pavone has nothing to disclose. F. Bonella reports grants and personal fees from Boehringer Ingelheim and Roche, and consultancy fees from BMS, Galapagos, GSK and Savara, outside the submitted work. V. Cottin reports personal fees and non-financial support from Actelion and Roche/Promedior; grants, personal fees and non-financial support from Boehringer Ingelheim; and personal fees from Bayer/MSD, Novartis, Sanofi, Celgene/ BMS, Galapagos, Galecto, Shionogi, AstraZeneca and Fibrogen, outside the submitted work. C. Valenzuela reports personal fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Galapagos and BMS, outside the submitted work. M. Wijsenbeek reports grants and other funding from Boehringer Ingelheim and Hoffman-La Roche, and other funding from Respivant, Galapagos, Novartis and Savara, outside the submitted work. All fees and grants were paid to her institution. E. Bendstrup reports grants and personal fees from Boehringer Ingelheim and Hoffmann-La Roche, and personal fees from Galapagos and AstraZeneca, outside the submitted work.

Published
2021

28. Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Author

Sebastiani, M, Luppi, F, Sambataro, G, Castillo Villegas, D, Cerri, S, Tomietto, P, Cassone, G, Bocchino, M, Atienza-Mateo, B, Cameli, P, Moya Alvarado, P, Faverio, P, Bargagli, E, Vancheri, C, Gonzalez-Gay, M, Clini, E, Salvarani, C, Manfredi, A, Sebastiani, Marco, Luppi, Fabrizio, Sambataro, Gianluca, Castillo Villegas, Diego, Cerri, Stefania, Tomietto, Paola, Cassone, Giulia, Bocchino, Marialuisa, Atienza-Mateo, Belen, Cameli, Paolo, Moya Alvarado, Patricia, Faverio, Paola, Bargagli, Elena, Vancheri, Carlo, Gonzalez-Gay, Miguel A, Clini, Enrico, Salvarani, Carlo, Manfredi, Andreina, Sebastiani, M, Luppi, F, Sambataro, G, Castillo Villegas, D, Cerri, S, Tomietto, P, Cassone, G, Bocchino, M, Atienza-Mateo, B, Cameli, P, Moya Alvarado, P, Faverio, P, Bargagli, E, Vancheri, C, Gonzalez-Gay, M, Clini, E, Salvarani, C, Manfredi, A, Sebastiani, Marco, Luppi, Fabrizio, Sambataro, Gianluca, Castillo Villegas, Diego, Cerri, Stefania, Tomietto, Paola, Cassone, Giulia, Bocchino, Marialuisa, Atienza-Mateo, Belen, Cameli, Paolo, Moya Alvarado, Patricia, Faverio, Paola, Bargagli, Elena, Vancheri, Carlo, Gonzalez-Gay, Miguel A, Clini, Enrico, Salvarani, Carlo, and Manfredi, Andreina

Abstract

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7-23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients' survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

Published
2021

29. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

Author

Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, Bendstrup, Elisabeth, Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, and Bendstrup, Elisabeth

Abstract

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Published
2021

30. Suggestions for lung function testing in the context of COVID-19

Author

Milanese, M., Corsico, A. G., Bellofiore, S., Carrozzi, L., Di Marco, F., Iovene, Bruno, Richeldi, Luca, Sanna, A., Santus, P., Schisano, M., Scichilone, N., Vancheri, C., Cerveri, I., Iovene B., Richeldi L. (ORCID:0000-0001-8594-1448), Milanese, M., Corsico, A. G., Bellofiore, S., Carrozzi, L., Di Marco, F., Iovene, Bruno, Richeldi, Luca, Sanna, A., Santus, P., Schisano, M., Scichilone, N., Vancheri, C., Cerveri, I., Iovene B., and Richeldi L. (ORCID:0000-0001-8594-1448)

Abstract

The 2019 coronavirus disease (COVID-19) pandemic is currently a challenge worldwide. Due to the characteristics of lung function tests, the risk of cross infection may be high between health care workers and patients. The role of lung function testing is well defined for the diagnosis of various diseases and conditions. Lung function tests are also indispensable in evaluating the response to medical treatment, in monitoring patient respiratory and systemic pathologies, and in evaluating preoperative risk in cardiothoracic and major abdominal surgeries. However, lung function testing represents a potential route for COVID-19 transmission, due to the aerosol generated during the procedures and the concentration of patients with pulmonary diseases in lung function laboratories. Currently, the opportunities for COVID-19 transmission remain partially unknown, and data are continuously evolving. This review provides useful information on the risks and recommendations for lung function testing, which have varied according to the phase of the pandemic. This information may support national and regional boards and the health authorities to which they belong. There is a need for rapid re-opening of lung function laboratories, but maximum safety is required in the COVID-19 era.

Published
2021

31. Cellular and molecular effects of protons: Apoptosis induction and potential implications for cancer therapy

Author

Di Pietro, C., Piro, S., Tabbì, G., Ragusa, M., Di Pietro, V., Zimmitti, V., Cuda, F., Anello, M., Consoli, U., Salinaro, E. T., Caruso, M., Vancheri, C., Crimi, N., Sabini, M. G., Cirrone, G. A. P., Raffaele, L., Privitera, G., Pulvirenti, A., Giugno, R., Ferro, A., Cuttone, G., Nigro, S. Lo, Purrello, R., Purrello, F., and Purrello, M.

Published
2006
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34. 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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35. 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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36. AB0528 CHARACTERIZATION OF ANTI-MPO POSITIVE INTERSTITIAL LUNG DISEASE. CLINICAL-SEROLOGIC AND RADIOLOGIC FEATURES AND SURVIVAL

Author

Cassone, G., primary, Dei, G., additional, Sambataro, G., additional, Manfredi, A., additional, Cerri, S., additional, Vacchi, C., additional, Faverio, P., additional, Sambataro, D., additional, Gozzi, F., additional, Vancheri, C., additional, Salvarani, C., additional, Luppi, F., additional, and Sebastiani, M., additional

Published
2020
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37. The DIAMORFOSIS (Diagnosis and Management of Lung Cancer and Fibrosis) Survey

Author

Tzouvelekis, A.E., primary, Antoniou, K., additional, Kreuter, M., additional, Evison, M., additional, Karampitsakos, T., additional, Blum, T., additional, Poletti, V., additional, Spagnolo, P., additional, Bonella, F., additional, Grigoriu, B.D., additional, Vancheri, C., additional, Cadranel, J., additional, and Bouros, D., additional

Published
2020
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38. 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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41. Acute exacerbation of idiopathic pulmonary fibrosis: International survey and call for harmonisation

Author

Kreuter, M. Polke, M. Walsh, S.L.F. Krisam, J. Collard, H.R. Chaudhuri, N. Avdeev, S. Behr, J. Calligaro, G. Corte, T. Flaherty, K. Funke-Chambour, M. Kolb, M. Kondoh, Y. Maher, T.M. Molina, M.M. Morais, A. Moor, C.C. Morisset, J. Pereira, C. Quadrelli, S. Selman, M. Tzouvelekis, A. Valenzuela, C. Vancheri, C. Vicens-Zygmunt, V. Wälscher, J. Wuyts, W. Wijsenbeek, M. Cottin, V. Bendstrup, E.

Subjects
respiratory system, humanities, respiratory tract diseases
Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. 509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%). Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed. Copyright © ERS 2020

Published
2020

42. consensus recommendations, and research priorities

Author

George, PM, Spagnolo, P, Kreuter, M, Altinisik, G, Bonifazi, M, Martinez, FJ, Molyneaux, PL, Renzoni, EA, Richeldi, L, Tomassetti, S, Valenzuela, C, Vancheri, C, Varone, F, Cottin, V, and Costabel, U

Abstract

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice. C1 [George, Peter M.; Molyneaux, Philip L.; Renzoni, Elisabetta A.] Royal Brompton & Harefield NHS Fdn Trust, Interstitial Lung Dis Unit, London, England. [George, Peter M.; Molyneaux, Philip L.; Renzoni, Elisabetta A.] Imperial Coll London, Natl Heart & Lung Inst, London, England. [Spagnolo, Paolo] Univ Padua, Dept Cardiac Thorac Vasc Sci & Publ Hlth, Padua, Italy. [Kreuter, Michael] Heidelberg Univ, Ctr Interstitial & Rare Lung Dis Pneumol, Thoraxklin, Heidelberg, Germany. [Kreuter, Michael] German Ctr Lung Res, Heidelberg, Germany. [Altinisik, Goksel] Pamukkale Univ, Fac Med, Dept Chest Dis, Denizli, Turkey. [Bonifazi, Martina] Univ Politecn Marche, Azienda Osped Univ Osped Riuniti, Resp Dis Unit, Dept Biomed Sci & Publ Hlth, Ancona, Italy. [Martinez, Fernando J.] Weill Cornell Med Coll, Dept Med, New York, NY USA. [Richeldi, Luca] Univ Cattolica Sacro Cuore, Fdn Policlin Univ A Gemelli IRCCS, Rome, Italy. [Tomassetti, Sara] Careggi Univ Hosp, Dept Expt & Clin Med, Florence, Italy. [Valenzuela, Claudia] Hosp Univ La Princesa, Inst Invest Princesa, Madrid, Spain. [Vancheri, Carlo] Univ Catania, Univ Hosp Policlin G Rodol, Reg Referral Ctr Rare Lung Dis, Dept Clin & Expt Med, Catania, Italy. [Varone, Francesco] Fdn Policlin A Gemelli IRCCS, Rome, Italy. [Cottin, Vincent] Hosp Civils Lyon, Louis Pradel Hosp, Natl Reference Coordinating Ctr Rare Pulm Dis, Dept Resp Med, Lyon, France. [Cottin, Vincent] Univ Lyon, Univ Claude Bernard Lyon 1, UMR754, IVPC, Lyon, France. [Costabel, Ulrich] Univ Duisburg Essen, Univ Hosp, Ruhrlandklin, Dept Pneumol, Essen, Germany.

Published
2020

43. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities

Author

George, P.M., Spagnolo, P., Kreuter, M., Altınışık, Göksel, Bonifazi, M., Martinez, F.J., Molyneaux, P.L., Renzoni, E.A., Richeldi, L., Tomassetti, S., Valenzuela, C., Vancheri, C., Varone, F., Cottin, V., Costabel, U., and Erice ILD working group

Subjects
antifibrotic activity, lung biopsy, Biomedical Research, evidence based medicine, phenotype, Pulmonary Fibrosis, prevalence, antifibrotic agent, Review, lysophosphatidic acid 1 receptor antagonist, medical research, allogeneic stem cell transplantation, forced vital capacity, nintedanib, Humans, human, multidisciplinary team, interstitial lung disease, research, disease predisposition, lung fibrosis, deep learning, consensus development, immunosuppressive treatment, lung function, patient risk, respiratory system, respiratory tract diseases, unclassified drug, clinical practice, fibrosing alveolitis, priority journal, risk factor, disease exacerbation, Disease Progression, medical decision making, mesenchymal stem cell transplantation, diagnostic accuracy, pathology, pirfenidone, Lung Diseases, Interstitial, bone marrow derived mesenchymal stem cell
Abstract

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice. © 2020 Elsevier Ltd

Published
2020

45. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities

Author

George, PM, Spagnolo, P, Kreuter, M, Altinisik, G, Bonifazi, M, Martinez, FJ, Molyneaux, PL, Renzoni, EA, Richeldi, L, Tomassetti, S, Valenzuela, C, Vancheri, C, Varone, F, Cottin, V, Costabel, U, Erice ILD working group, The Jon Moulton Charity Trust, and Action for Pulmonary Fibrosis

Subjects
Pulmonary and Respiratory Medicine, Lung Diseases, medicine.medical_specialty, Biomedical Research, Pulmonary Fibrosis, Erice ILD working group, Medizin, Settore MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, Multidisciplinary team, 1117 Public Health and Health Services, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Fibrosis, Pulmonary fibrosis, Medicine, Humans, 030212 general & internal medicine, Intensive care medicine, Lung, business.industry, Research, Interstitial lung disease, 1103 Clinical Sciences, respiratory system, medicine.disease, respiratory tract diseases, Clinical Practice, medicine.anatomical_structure, Disease Progression, Lung Diseases, Interstitial, 030228 respiratory system, Position paper, business, Interstitial, 1199 Other Medical and Health Sciences
Abstract

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice.

Published
2020

49. Acute exacerbation of idiopathic pulmonary fibrosis

Author

Kreuter, M. (Michael), Polke, M. (Markus), Walsh, S.L.F. (Simon L.F.), Krisam, J. (Johannes), Collard, H.R. (Harold R.), Chaudhuri, N. (Nazia), Avdeev, S. (Sergey), Behr, J. (Jürgen), Calligaro, G. (Gregory), Corte, T. (Tamera), Flaherty, K. (Kevin), Funke-Chambour, M. (Manuela), Kolb, M. (Martin), Kondoh, Y. (Yasuhiro), Maher, T.M. (Toby M.), Molina, M.M. (Maria Molina), Morais, A. (Antonio), Moor, C.C. (Karen), Morisset, J. (Julie), Pereira, C. (Carlos), Quadrelli, S. (Silvia), Selman, M. (Moises), Tzouvelekis, A. (Argyrios), Valenzuela, C. (Claudia), Vancheri, C. (Carlo), Vicens-Zygmunt, V. (Vanesa), Wälscher, J. (Julia), Wuyts, W.A. (Wim A.), Wijsenbeek-Lourens, M.S. (Marlies), Cottin, V. (Vincent), Bendstrup, E. (Elisabeth), Kreuter, M. (Michael), Polke, M. (Markus), Walsh, S.L.F. (Simon L.F.), Krisam, J. (Johannes), Collard, H.R. (Harold R.), Chaudhuri, N. (Nazia), Avdeev, S. (Sergey), Behr, J. (Jürgen), Calligaro, G. (Gregory), Corte, T. (Tamera), Flaherty, K. (Kevin), Funke-Chambour, M. (Manuela), Kolb, M. (Martin), Kondoh, Y. (Yasuhiro), Maher, T.M. (Toby M.), Molina, M.M. (Maria Molina), Morais, A. (Antonio), Moor, C.C. (Karen), Morisset, J. (Julie), Pereira, C. (Carlos), Quadrelli, S. (Silvia), Selman, M. (Moises), Tzouvelekis, A. (Argyrios), Valenzuela, C. (Claudia), Vancheri, C. (Carlo), Vicens-Zygmunt, V. (Vanesa), Wälscher, J. (Julia), Wuyts, W.A. (Wim A.), Wijsenbeek-Lourens, M.S. (Marlies), Cottin, V. (Vincent), and Bendstrup, E. (Elisabeth)

Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. 509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%). Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed.

Published
2020
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50. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities

Author

George, P. M., Spagnolo, P., Kreuter, M., Altinisik, G., Bonifazi, M., Martinez, F. J., Molyneaux, P. L., Renzoni, E. A., Richeldi, Luca, Tomassetti, S., Valenzuela, C., Vancheri, C., Varone, Francesco, Cottin, V., Costabel, U., Richeldi L. (ORCID:0000-0001-8594-1448), Varone F., George, P. M., Spagnolo, P., Kreuter, M., Altinisik, G., Bonifazi, M., Martinez, F. J., Molyneaux, P. L., Renzoni, E. A., Richeldi, Luca, Tomassetti, S., Valenzuela, C., Vancheri, C., Varone, Francesco, Cottin, V., Costabel, U., Richeldi L. (ORCID:0000-0001-8594-1448), and Varone F.

Abstract

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice.

Published
2020

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