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3. 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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6. Loss of muscle mass in Idiopathic Pulmonary Fibrosis

Author

Bernardinello, N, primary, Petrarulo, S, additional, Grisostomi, G, additional, Modugno, A, additional, Bertin, S, additional, Pasin, D, additional, Cocconcelli, E, additional, Spagnolo, P, additional, Giraudo, C, additional, and Balestro, E, additional

Published
2022
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9. Lung Cancer in Patients with Idiopathic Pulmonary Fibrosis: A Retrospective Multicenter Study in Europe

Author

Karampitsakos, T., primary, Spagnolo, P., additional, Mogulkoc, N., additional, Wuyts, W.A., additional, Tomassetti, S., additional, Bendstrup, E., additional, Molina Molina, M., additional, Manali, E., additional, Unat, O.S., additional, Kahn, N., additional, Kolilekas, L., additional, Rosi, E., additional, Gori, L., additional, Ravaglia, C., additional, Daniil, Z., additional, Prior, T.S., additional, Papanikolaou, I.C., additional, Aso, S., additional, Tryfon, S., additional, Papakosta, D., additional, Balestro, E., additional, Papiris, S., additional, Antoniou, K., additional, Bouros, D.E., additional, Wells, A.U., additional, Kreuter, M., additional, and Tzouvelekis, A.E., additional

Published
2022
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10. Comorbidities, cardiovascular therapies, and COVID-19 mortality: A nationwide, italian observational study (ItaliCO)

Author

Polverino, F, Stern, D, Ruocco, G, Balestro, E, Bassetti, M, Candelli, M, Cirillo, B, Contoli, M, Corsico, A, D'Amico, F, D'Elia, E, Falco, G, Gasparini, S, Guerra, S, Harari, S, Kraft, M, Mennella, L, Papi, A, Parrella, R, Pelosi, P, Poletti, V, Polverino, M, Tana, C, Terribile, R, Woods, J, Di Marco, F, Martinez, F, Zhang, S, Geelhoed, B, Sinning, C, Agarossi, A, Agati, S, Agosteo, E, Ando', F, Andreoni, M, Angelillo, I, Arcoleo, G, Arena, C, Baiamonte, P, Ball, L, Banfi, P, Bartoletti, G, Bartolotta, R, Battaglini, D, Bellan, M, Benzoni, I, Bertolini, R, Bevilacqua, M, Bezzi, M, Bianco, A, Bisbano, A, Bobbio, F, Bocchialini, G, Bonetti, F, Boni, F, Bonifazi, M, Borgonovo, G, Borre', S, Bosio, M, Brachini, G, Brunetti, I, Calagna, L, Calo, F, Capuozzo, A, Carr, T, Castellani, A, Catalano, F, Catania, G, Catena, E, Cattaneo, M, Cattelan, A, Ceruti, V, Chiumiento, F, Cicchitto, G, Confalonieri, M, Confalonieri, P, Coppola, N, Cosentina, R, Costantino, R, Crimi, C, Curra, A, D'Abbraccio, M, Dalbeni, A, Daleffe, F, Davide, R, Del Donno, M, Di Pastena, F, Di Perna, F, Di Rosa, Z, Di Sabatino, A, Elesbani, O, Elia, D, Esposito, V, Fabiani, L, Falo, G, Fanelli, C, Fantin, A, Ferrigno, F, Fiorentino, G, Franceschi, F, Fronza, M, Gardenghi, G, Giacobbe, D, Giannotti, C, Giannotti, G, Gidari, A, Giovanardi, F, Gnerre, P, Gonnelli, F, Graziano, M, Greco, S, Grosso, A, Guarino, S, Iannarelli, A, Imitazione, P, Inglese, F, Iodice, V, Izzo, A, La Greca, C, Lax, A, Legittimo, F, Leo, A, Leone, S, Lepidini, V, Leto, M, Licata, F, Locati, F, Lorini, L, Lucchetti, B, Maida, I, Macera, M, Manzillo, E, March, A, Mascheroni, D, Mastroianni, A, Mauro, I, Mazzitelli, M, Mazzuca, E, Micheletto, C, Mingoli, A, Minuz, P, Moioli, M, Monti, L, Morgagni, R, Mucci, L, Muselli, M, Negri, S, Nobile, C, Oldani, S, Olivieri, C, Parati, G, Parodi, L, Pastorelli, E, Patruno, V, Pellegrino, F, Pengo, M, Pepe, D, Perotti, A, Petrino, R, Petrucci, M, Piane, R, Pignataro, G, Pino, M, Pirisi, M, Porru, F, Pugliese, F, Punzi, R, Ramaroli, D, Robba, C, Rostagno, R, Sabatini, U, Sainaghi, P, Salton, F, Salzano, C, Sanduzzi, A, Zamparelli, S, Sangiovanni, V, Santopuoli, D, Sapienza, P, Sarmati, L, Schiaroli, E, Scienza, F, Senni, M, Serchisu, L, Sgherzi, S, Soddu, D, Soranna, D, Sorino, C, Spadaro, S, Stirpe, E, Tardivo, S, Tartaglia, S, Teopompi, E, Tomchaney, M, Torelli, E, Torlasco, C, Torti, C, Tupputi, E, Ugolinelli, C, Vatrella, A, Versace, A, Villani, M, Vincenzo, L, Volta, C, Voraphani, N, Zekaj, E, Zoppellari, R, Polverino F., Stern D. A., Ruocco G., Balestro E., Bassetti M., Candelli M., Cirillo B., Contoli M., Corsico A., D'Amico F., D'Elia E., Falco G., Gasparini S., Guerra S., Harari S., Kraft M., Mennella L., Papi A., Parrella R., Pelosi P., Poletti V., Polverino M., Tana C., Terribile R., Woods J. C., Di Marco F., Martinez F. D., Zhang S., Geelhoed B., Sinning C., Agarossi A., Agati S., Agosteo E., Ando' F., Andreoni M., Angelillo I. F., Arcoleo G., Arena C., Baiamonte P., Ball L., Banfi P., Bartoletti G., Bartolotta R., Battaglini D., Bellan M., Benzoni I., Bertolini R., Bevilacqua M., Bezzi M., Bianco A., Bisbano A., Bobbio F., Bocchialini G., Bonetti F., Boni F., Bonifazi M., Borgonovo G., Borre' S., Bosio M., Brachini G., Brunetti I., Calagna L., Calo F., Capuozzo A., Carr T., Castellani A., Catalano F., Catania G., Catena E., Cattaneo M., Cattelan A., Ceruti V., Chiumiento F., Cicchitto G., Confalonieri M., Confalonieri P., Coppola N., Cosentina R., Costantino R., Crimi C., Curra A., D'Abbraccio M., Dalbeni A., Daleffe F., Davide R., Del Donno M., Di Pastena F., Di Perna F., Di Rosa Z., Di Sabatino A., Elesbani O., Elia D., Esposito V., Fabiani L., Falo G., Fanelli C., Fantin A., Ferrigno F., Fiorentino G., Franceschi F., Fronza M., Gardenghi G. G., Giacobbe D. R., Giannotti C., Giannotti G., Gidari A., Giovanardi F., Gnerre P., Gonnelli F., Graziano M., Greco S., Grosso A., Guarino S., Iannarelli A., Imitazione P., Inglese F., Iodice V., Izzo A., La Greca C., Lax A., Legittimo F., Leo A., Leone S., Lepidini V., Leto M., Licata F., Locati F., Lorini L., Lucchetti B., Maida I., Macera M., Manzillo E., March A., Mascheroni D., Mastroianni A., Mauro I., Mazzitelli M., Mazzuca E., Micheletto C., Mingoli A., Minuz P., Moioli M., Monti L., Morgagni R., Mucci L., Muselli M., Negri S., Nobile C. G. A., Oldani S., Olivieri C., Parati G., Parodi L., Pastorelli E., Patruno V., Pellegrino F., Pengo M. F., Pepe D., Perotti A., Petrino R., Petrucci M., Piane R. M., Pignataro G., Pino M., Pirisi M., Porru F., Pugliese F., Punzi R., Ramaroli D. A., Robba C., Rostagno R., Sabatini U., Sainaghi P. P., Salton F., Salzano C., Sanduzzi A., Zamparelli S. S., Sangiovanni V., Santopuoli D., Sapienza P., Sarmati L., Schiaroli E., Scienza F., Senni M., Serchisu L., Sgherzi S., Soddu D., Soranna D., Sorino C., Spadaro S., Stirpe E., Tardivo S., Tartaglia S., Teopompi E., Tomchaney M., Torelli E., Torlasco C., Torti C., Tupputi E., Ugolinelli C., Vatrella A., Versace A. G., Villani M., Vincenzo L., Volta C. A., Voraphani N., Zekaj E., Zoppellari R., Polverino, F, Stern, D, Ruocco, G, Balestro, E, Bassetti, M, Candelli, M, Cirillo, B, Contoli, M, Corsico, A, D'Amico, F, D'Elia, E, Falco, G, Gasparini, S, Guerra, S, Harari, S, Kraft, M, Mennella, L, Papi, A, Parrella, R, Pelosi, P, Poletti, V, Polverino, M, Tana, C, Terribile, R, Woods, J, Di Marco, F, Martinez, F, Zhang, S, Geelhoed, B, Sinning, C, Agarossi, A, Agati, S, Agosteo, E, Ando', F, Andreoni, M, Angelillo, I, Arcoleo, G, Arena, C, Baiamonte, P, Ball, L, Banfi, P, Bartoletti, G, Bartolotta, R, Battaglini, D, Bellan, M, Benzoni, I, Bertolini, R, Bevilacqua, M, Bezzi, M, Bianco, A, Bisbano, A, Bobbio, F, Bocchialini, G, Bonetti, F, Boni, F, Bonifazi, M, Borgonovo, G, Borre', S, Bosio, M, Brachini, G, Brunetti, I, Calagna, L, Calo, F, Capuozzo, A, Carr, T, Castellani, A, Catalano, F, Catania, G, Catena, E, Cattaneo, M, Cattelan, A, Ceruti, V, Chiumiento, F, Cicchitto, G, Confalonieri, M, Confalonieri, P, Coppola, N, Cosentina, R, Costantino, R, Crimi, C, Curra, A, D'Abbraccio, M, Dalbeni, A, Daleffe, F, Davide, R, Del Donno, M, Di Pastena, F, Di Perna, F, Di Rosa, Z, Di Sabatino, A, Elesbani, O, Elia, D, Esposito, V, Fabiani, L, Falo, G, Fanelli, C, Fantin, A, Ferrigno, F, Fiorentino, G, Franceschi, F, Fronza, M, Gardenghi, G, Giacobbe, D, Giannotti, C, Giannotti, G, Gidari, A, Giovanardi, F, Gnerre, P, Gonnelli, F, Graziano, M, Greco, S, Grosso, A, Guarino, S, Iannarelli, A, Imitazione, P, Inglese, F, Iodice, V, Izzo, A, La Greca, C, Lax, A, Legittimo, F, Leo, A, Leone, S, Lepidini, V, Leto, M, Licata, F, Locati, F, Lorini, L, Lucchetti, B, Maida, I, Macera, M, Manzillo, E, March, A, Mascheroni, D, Mastroianni, A, Mauro, I, Mazzitelli, M, Mazzuca, E, Micheletto, C, Mingoli, A, Minuz, P, Moioli, M, Monti, L, Morgagni, R, Mucci, L, Muselli, M, Negri, S, Nobile, C, Oldani, S, Olivieri, C, Parati, G, Parodi, L, Pastorelli, E, Patruno, V, Pellegrino, F, Pengo, M, Pepe, D, Perotti, A, Petrino, R, Petrucci, M, Piane, R, Pignataro, G, Pino, M, Pirisi, M, Porru, F, Pugliese, F, Punzi, R, Ramaroli, D, Robba, C, Rostagno, R, Sabatini, U, Sainaghi, P, Salton, F, Salzano, C, Sanduzzi, A, Zamparelli, S, Sangiovanni, V, Santopuoli, D, Sapienza, P, Sarmati, L, Schiaroli, E, Scienza, F, Senni, M, Serchisu, L, Sgherzi, S, Soddu, D, Soranna, D, Sorino, C, Spadaro, S, Stirpe, E, Tardivo, S, Tartaglia, S, Teopompi, E, Tomchaney, M, Torelli, E, Torlasco, C, Torti, C, Tupputi, E, Ugolinelli, C, Vatrella, A, Versace, A, Villani, M, Vincenzo, L, Volta, C, Voraphani, N, Zekaj, E, Zoppellari, R, Polverino F., Stern D. A., Ruocco G., Balestro E., Bassetti M., Candelli M., Cirillo B., Contoli M., Corsico A., D'Amico F., D'Elia E., Falco G., Gasparini S., Guerra S., Harari S., Kraft M., Mennella L., Papi A., Parrella R., Pelosi P., Poletti V., Polverino M., Tana C., Terribile R., Woods J. C., Di Marco F., Martinez F. D., Zhang S., Geelhoed B., Sinning C., Agarossi A., Agati S., Agosteo E., Ando' F., Andreoni M., Angelillo I. F., Arcoleo G., Arena C., Baiamonte P., Ball L., Banfi P., Bartoletti G., Bartolotta R., Battaglini D., Bellan M., Benzoni I., Bertolini R., Bevilacqua M., Bezzi M., Bianco A., Bisbano A., Bobbio F., Bocchialini G., Bonetti F., Boni F., Bonifazi M., Borgonovo G., Borre' S., Bosio M., Brachini G., Brunetti I., Calagna L., Calo F., Capuozzo A., Carr T., Castellani A., Catalano F., Catania G., Catena E., Cattaneo M., Cattelan A., Ceruti V., Chiumiento F., Cicchitto G., Confalonieri M., Confalonieri P., Coppola N., Cosentina R., Costantino R., Crimi C., Curra A., D'Abbraccio M., Dalbeni A., Daleffe F., Davide R., Del Donno M., Di Pastena F., Di Perna F., Di Rosa Z., Di Sabatino A., Elesbani O., Elia D., Esposito V., Fabiani L., Falo G., Fanelli C., Fantin A., Ferrigno F., Fiorentino G., Franceschi F., Fronza M., Gardenghi G. G., Giacobbe D. R., Giannotti C., Giannotti G., Gidari A., Giovanardi F., Gnerre P., Gonnelli F., Graziano M., Greco S., Grosso A., Guarino S., Iannarelli A., Imitazione P., Inglese F., Iodice V., Izzo A., La Greca C., Lax A., Legittimo F., Leo A., Leone S., Lepidini V., Leto M., Licata F., Locati F., Lorini L., Lucchetti B., Maida I., Macera M., Manzillo E., March A., Mascheroni D., Mastroianni A., Mauro I., Mazzitelli M., Mazzuca E., Micheletto C., Mingoli A., Minuz P., Moioli M., Monti L., Morgagni R., Mucci L., Muselli M., Negri S., Nobile C. G. A., Oldani S., Olivieri C., Parati G., Parodi L., Pastorelli E., Patruno V., Pellegrino F., Pengo M. F., Pepe D., Perotti A., Petrino R., Petrucci M., Piane R. M., Pignataro G., Pino M., Pirisi M., Porru F., Pugliese F., Punzi R., Ramaroli D. A., Robba C., Rostagno R., Sabatini U., Sainaghi P. P., Salton F., Salzano C., Sanduzzi A., Zamparelli S. S., Sangiovanni V., Santopuoli D., Sapienza P., Sarmati L., Schiaroli E., Scienza F., Senni M., Serchisu L., Sgherzi S., Soddu D., Soranna D., Sorino C., Spadaro S., Stirpe E., Tardivo S., Tartaglia S., Teopompi E., Tomchaney M., Torelli E., Torlasco C., Torti C., Tupputi E., Ugolinelli C., Vatrella A., Versace A. G., Villani M., Vincenzo L., Volta C. A., Voraphani N., Zekaj E., and Zoppellari R.

Abstract

Background: Italy has one of the world’s oldest populations, and suffered one the highest death tolls from Coronavirus disease 2019 (COVID-19) worldwide. Older people with cardiovascular diseases (CVDs), and in particular hypertension, are at higher risk of hospitalization and death for COVID-19. Whether hypertensionmedicationsmay increase the risk for death in older COVID 19 inpatients at the highest risk for the disease is currently unknown. Methods: Data from 5,625 COVID-19 inpatients were manually extracted from medical charts from 61 hospitals across Italy. From the initial 5,625 patients, 3,179 were included in the study as they were either discharged or deceased at the time of the data analysis. Primary outcome was inpatient death or recovery. Mixed effects logistic regression models were adjusted for sex, age, and number of comorbidities, with a random effect for site. Results: A large proportion of participating inpatients were ≥65 years old (58%), male (68%), non-smokers (93%) with comorbidities (66%). Each additional comorbidity increased the risk of death by 35% [adjOR = 1.35 (1.2, 1.5) p < 0.001]. Use of ACE inhibitors, ARBs, beta-blockers or Ca-antagonists was not associated with significantly increased risk of death. There was a marginal negative association between ARB use and death, and a marginal positive association between diuretic use and death. Conclusions: This Italian nationwide observational study of COVID-19 inpatients, the majority of which ≥65 years old, indicates that there is a linear direct relationship between the number of comorbidities and the risk of death. Among CVDs, hypertension and pre-existing cardiomyopathy were significantly associated with risk of death. The use of hypertension medications reported to be safe in younger cohorts, do not contribute significantly to increased COVID-19 related deaths in an older population that suffered one of the highest death tolls worldwide.

Published
2020

11. Comorbidities, cardiovascular therapies, and COVID-19 mortality: A nationwide, italian observational study (ItaliCO)

Author

Polverino, F., Phd, Md, Stern, D., Polverino, M., D'Amico, F., D'Elia, E., Agarossi, A., Agati, S., Agosteo, E., Ando', F., Andreoni, M., Angelillo, If., Dds, Mph, Arcoleo, G., Arena, C., Baiamonte, P., Balestro, E., Ball, L., Banfi, P., Bartoletti, G., Bartolotta, R., Bassetti, M., Battaglini, D., Bellan, M., Benzoni, I., Bertolini, R., Bevilacqua, M., Bezzi, M., Bianco, A., Bisbano, A., Bobbio, F., Bocchialini, G., Bonetti, F., Boni, F., Bonifazi, M., Borgonovo, G., Borre', S., Bosio, M., Brachini, G., Brunetti, I., Calagna, L., Calò, F., Candelli, M., Capuozzo, A., Carr, T., Castellani, A., Catalano, F., Catania, G., Catena, E., Cattaneo, M., Cattelan, A., Ceruti, V., Chiumiento, F., Cicchitto, G., Cirillo, B., Confalonieri, M., Confalonieri, P., Contoli, M., Coppola, N., Corsico, A., Cosentina, R., Costantino, R., Crimi, C., Currà, A., D'Abbraccio, M., Dalbeni, A., Daleffe, F., Davide, R., Del Donno, M., Di Marco, F., Di Pastena, F., Di Perna, F., Di Rosa, Z., Di Sabatino, A., Elesbani, O., Elia, D., Esposito, V., Fabiani, L., Falco, G., Falo, G., Fanelli, C., Fantin, A., Ferrigno, F., Fiorentino, G., Franceschi, F., Fronza, M., Gardini Gardenghi, G., Gasparini, S., Giacobbe, D. R., Giannotti, C., Giannotti, G., Gidari, A., Giovanardi, F., Gnerre, P., Gonnelli, F., Graziano, M., Greco, S., Grosso, A., Phd, Guarino, S., Guerra, S., Harari, S., Iannarelli, A., Imitazione, P., Inglese, F., Iodice, V., Izzo, A., La Greca, C., Kraft, M., Lax, A., Legittimo, F., Leo, A., Leone, S., Lepidini, V., Leto, M., Licata, F., Locati, F., Lorini, L., Lucchetti, B., Maida, I., Macera, M., Manzillo, E., March, A., Mascheroni, D., Mastroianni, A., Mauro, I., Mazzitelli, M., Mazzuca, E., Mennella, L., Micheletto, C., Mingoli, A., Minuz, P., Moioli, M., Monti, L., Morgagni, R., Mucci, L., Muselli, M., Negri, S., Nobile, C. G. A., Oldani, S., Olivieri, C., Papi, A., Parati, G., Parodi, L., Parrella, R., Pastorelli, E., Patruno, V., Pellegrino, F., Pelosi, P., Fers, Md, Pengo, M. F., Pepe, D., Perotti, A., Petrino, R., Petrucci, M., Piane, R. M., Pignataro, G., Pino, M., Pirisi, M., Poletti, V., Porru, F., Pugliese, F., Punzi, R., Ramaroli, D. A., Robba, C., Rostagno, R., Ruocco, G., Sabatini, U., Sainaghi, P. P., Salton, F., Salzano, C., Sanduzzi, A., Sanduzzi Zamparelli, S., Sangiovanni, V., Santopuoli, D., Sapienza, P., Sarmati, L., Schiaroli, E., Scienza, F., Senni, M., Serchisu, L., Sgherzi, S., Soddu, D., Soranna, D., Sorino, C., Spadaro, S., Stirpe, E., Tana, C., Tardivo, S., Tartaglia, S., Teopompi, E., Terribile, R., Tomchaney, M., Torelli, E., Torlasco, C., Torti, C., Tupputi, E., Ugolinelli, C., Vatrella, A., Versace, A. G., Villani, M., Vincenzo, L., Volta, C. A., Voraphani, N., Woods, J. C., Zekaj, E., Zoppellari, R., Martinez, and F. D., Polverino, Francesca, Stern, Debra A, Ruocco, Gaetano, Balestro, Elisabetta, Bassetti, Matteo, Candelli, Marcello, Cirillo, Bruno, Contoli, Marco, Corsico, Angelo, D'Amico, Filippo, D'Elia, Emilia, Falco, Giuseppe, Gasparini, Stefano, Guerra, Stefano, Harari, Sergio, Kraft, Monica, Mennella, Luigi, Papi, Alberto, Parrella, Roberto, Pelosi, Paolo, Poletti, Venerino, Polverino, Mario, Tana, Claudio, Terribile, Roberta, Woods, Jason C, Di Marco, Fabiano, Martinez, Fernando D, Angelillo, Italo Francesco, Stern, Debra A., C Woods, Jason, Martinez, Fernando D., Polverino, F., Stern, D., Polverino, M., D'Amico, F., D'Elia, E., Agarossi, A., Agati, S., Agosteo, E., Ando', F., Andreoni, M., Angelillo, If., Arcoleo, G., Arena, C., Baiamonte, P., Balestro, E., Ball, L., Banfi, P., Bartoletti, G., Bartolotta, R., Bassetti, M., Battaglini, D., Bellan, M., Benzoni, I., Bertolini, R., Bevilacqua, M., Bezzi, M., Bianco, A., Bisbano, A., Bobbio, F., Bocchialini, G., Bonetti, F., Boni, F., Bonifazi, M., Borgonovo, G., Borre', S., Bosio, M., Brachini, G., Brunetti, I., Calagna, L., Calò, F., Candelli, M., Capuozzo, A., Carr, T., Castellani, A., Catalano, F., Catania, G., Catena, E., Cattaneo, M., Cattelan, A., Ceruti, V., Chiumiento, F., Cicchitto, G., Cirillo, B., Confalonieri, M., Confalonieri, P., Contoli, M., Coppola, N., Corsico, A., Cosentina, R., Costantino, R., Crimi, C., Currà, A., D'Abbraccio, M., Dalbeni, A., Daleffe, F., Davide, R., Del Donno, M., Di Marco, F., Di Pastena, F., Di Perna, F., Di Rosa, Z., Di Sabatino, A., Elesbani, O., Elia, D., Esposito, V., Fabiani, L., Falco, G., Falo, G., Fanelli, C., Fantin, A., Ferrigno, F., Fiorentino, G., Franceschi, F., Fronza, M., Gardini Gardenghi, G., Gasparini, S., Giacobbe, D. R., Giannotti, C., Giannotti, G., Gidari, A., Giovanardi, F., Gnerre, P., Gonnelli, F., Graziano, M., Greco, S., Grosso, A., Guarino, S., Guerra, S., Harari, S., Iannarelli, A., Imitazione, P., Inglese, F., Iodice, V., Izzo, A., La Greca, C., Kraft, M., Lax, A., Legittimo, F., Leo, A., Leone, S., Lepidini, V., Leto, M., Licata, F., Locati, F., Lorini, L., Lucchetti, B., Maida, I., Macera, M., Manzillo, E., March, A., Mascheroni, D., Mastroianni, A., Mauro, I., Mazzitelli, M., Mazzuca, E., Mennella, L., Micheletto, C., Mingoli, A., Minuz, P., Moioli, M., Monti, L., Morgagni, R., Mucci, L., Muselli, M., Negri, S., Nobile, C. G. A., Oldani, S., Olivieri, C., Papi, A., Parati, G., Parodi, L., Parrella, R., Pastorelli, E., Patruno, V., Pellegrino, F., Pelosi, P., Pengo, M. F., Pepe, D., Perotti, A., Petrino, R., Petrucci, M., Piane, R. M., Pignataro, G., Pino, M., Pirisi, M., Poletti, V., Porru, F., Pugliese, F., Punzi, R., Ramaroli, D. A., Robba, C., Rostagno, R., Ruocco, G., Sabatini, U., Sainaghi, P. P., Salton, F., Salzano, C., Sanduzzi, A., Sanduzzi Zamparelli, S., Sangiovanni, V., Santopuoli, D., Sapienza, P., Sarmati, L., Schiaroli, E., Scienza, F., Senni, M., Serchisu, L., Sgherzi, S., Soddu, D., Soranna, D., Sorino, C., Spadaro, S., Stirpe, E., Tana, C., Tardivo, S., Tartaglia, S., Teopompi, E., Terribile, R., Tomchaney, M., Torelli, E., Torlasco, C., Torti, C., Tupputi, E., Ugolinelli, C., Vatrella, A., Versace, A. G., Villani, M., Vincenzo, L., Volta, C. A., Voraphani, N., Woods, J. C., Zekaj, E., Zoppellari, R., Martinez, F. D., Public Health, Polverino, F, Stern, D, Ruocco, G, Balestro, E, Bassetti, M, Candelli, M, Cirillo, B, Contoli, M, Corsico, A, D'Amico, F, D'Elia, E, Falco, G, Gasparini, S, Guerra, S, Harari, S, Kraft, M, Mennella, L, Papi, A, Parrella, R, Pelosi, P, Poletti, V, Polverino, M, Tana, C, Terribile, R, Woods, J, Di Marco, F, Martinez, F, Zhang, S, Geelhoed, B, Sinning, C, Agarossi, A, Agati, S, Agosteo, E, Ando', F, Andreoni, M, Angelillo, I, Arcoleo, G, Arena, C, Baiamonte, P, Ball, L, Banfi, P, Bartoletti, G, Bartolotta, R, Battaglini, D, Bellan, M, Benzoni, I, Bertolini, R, Bevilacqua, M, Bezzi, M, Bianco, A, Bisbano, A, Bobbio, F, Bocchialini, G, Bonetti, F, Boni, F, Bonifazi, M, Borgonovo, G, Borre', S, Bosio, M, Brachini, G, Brunetti, I, Calagna, L, Calo, F, Capuozzo, A, Carr, T, Castellani, A, Catalano, F, Catania, G, Catena, E, Cattaneo, M, Cattelan, A, Ceruti, V, Chiumiento, F, Cicchitto, G, Confalonieri, M, Confalonieri, P, Coppola, N, Cosentina, R, Costantino, R, Crimi, C, Curra, A, D'Abbraccio, M, Dalbeni, A, Daleffe, F, Davide, R, Del Donno, M, Di Pastena, F, Di Perna, F, Di Rosa, Z, Di Sabatino, A, Elesbani, O, Elia, D, Esposito, V, Fabiani, L, Falo, G, Fanelli, C, Fantin, A, Ferrigno, F, Fiorentino, G, Franceschi, F, Fronza, M, Gardenghi, G, Giacobbe, D, Giannotti, C, Giannotti, G, Gidari, A, Giovanardi, F, Gnerre, P, Gonnelli, F, Graziano, M, Greco, S, Grosso, A, Guarino, S, Iannarelli, A, Imitazione, P, Inglese, F, Iodice, V, Izzo, A, La Greca, C, Lax, A, Legittimo, F, Leo, A, Leone, S, Lepidini, V, Leto, M, Licata, F, Locati, F, Lorini, L, Lucchetti, B, Maida, I, Macera, M, Manzillo, E, March, A, Mascheroni, D, Mastroianni, A, Mauro, I, Mazzitelli, M, Mazzuca, E, Micheletto, C, Mingoli, A, Minuz, P, Moioli, M, Monti, L, Morgagni, R, Mucci, L, Muselli, M, Negri, S, Nobile, C, Oldani, S, Olivieri, C, Parati, G, Parodi, L, Pastorelli, E, Patruno, V, Pellegrino, F, Pengo, M, Pepe, D, Perotti, A, Petrino, R, Petrucci, M, Piane, R, Pignataro, G, Pino, M, Pirisi, M, Porru, F, Pugliese, F, Punzi, R, Ramaroli, D, Robba, C, Rostagno, R, Sabatini, U, Sainaghi, P, Salton, F, Salzano, C, Sanduzzi, A, Zamparelli, S, Sangiovanni, V, Santopuoli, D, Sapienza, P, Sarmati, L, Schiaroli, E, Scienza, F, Senni, M, Serchisu, L, Sgherzi, S, Soddu, D, Soranna, D, Sorino, C, Spadaro, S, Stirpe, E, Tardivo, S, Tartaglia, S, Teopompi, E, Tomchaney, M, Torelli, E, Torlasco, C, Torti, C, Tupputi, E, Ugolinelli, C, Vatrella, A, Versace, A, Villani, M, Vincenzo, L, Volta, C, Voraphani, N, Zekaj, E, and Zoppellari, R

Subjects
0301 basic medicine, COVID-19, comorbidities, ACE inhibitors, mortality, cohort study, medicine.medical_specialty, comorbiditie, lcsh:Diseases of the circulatory (Cardiovascular) system, ACE inhibitors, Coronavirus disease 2019 (COVID-19), COVID-19, cohort study, comorbidities, mortality, Cardiomyopathy, Socio-culturale, Disease, 030204 cardiovascular system & hematology, Cardiovascular Medicine, Logistic regression, Older population, Comorbidities, 03 medical and health sciences, 0302 clinical medicine, SDG 3 - Good Health and Well-being, Internal medicine, ACE inhibitor, medicine, Mortality, Original Research, business.industry, Cohort study, medicine.disease, Comorbidity, 030104 developmental biology, lcsh:RC666-701, Observational study, Erratum, Cardiology and Cardiovascular Medicine, business
Abstract

Background: Italy has one of the world’s oldest populations, and suffered one the highest death tolls from Coronavirus disease 2019 (COVID-19) worldwide. Older people with cardiovascular diseases (CVDs), and in particular hypertension, are at higher risk of hospitalization and death for COVID-19. Whether hypertensionmedicationsmay increase the risk for death in older COVID 19 inpatients at the highest risk for the disease is currently unknown. Methods: Data from 5,625 COVID-19 inpatients were manually extracted from medical charts from 61 hospitals across Italy. From the initial 5,625 patients, 3,179 were included in the study as they were either discharged or deceased at the time of the data analysis. Primary outcome was inpatient death or recovery. Mixed effects logistic regression models were adjusted for sex, age, and number of comorbidities, with a random effect for site. Results: A large proportion of participating inpatients were ≥65 years old (58%), male (68%), non-smokers (93%) with comorbidities (66%). Each additional comorbidity increased the risk of death by 35% [adjOR = 1.35 (1.2, 1.5) p < 0.001]. Use of ACE inhibitors, ARBs, beta-blockers or Ca-antagonists was not associated with significantly increased risk of death. There was a marginal negative association between ARB use and death, and a marginal positive association between diuretic use and death. Conclusions: This Italian nationwide observational study of COVID-19 inpatients, the majority of which ≥65 years old, indicates that there is a linear direct relationship between the number of comorbidities and the risk of death. Among CVDs, hypertension and pre-existing cardiomyopathy were significantly associated with risk of death. The use of hypertension medications reported to be safe in younger cohorts, do not contribute significantly to increased COVID-19 related deaths in an older population that suffered one of the highest death tolls worldwide.

Published
2020

14. 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

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, 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

16. Subclinical liver fibrosis in patients with idiopathic pulmonary fibrosis

Author

Cocconcelli, E, Tonelli, R, Abbati, G, Marchioni, A, Castaniere, I, Pelizzaro, F, Russo, F, Vegetti, A, Balestro, E, Pietrangelo, A, Richeldi, L, Luppi, F, Spagnolo, P, Clini, E, Cerri, S, Cocconcelli, Elisabetta, Tonelli, Roberto, Abbati, Gianluca, Marchioni, Alessandro, Castaniere, Ivana, Pelizzaro, Filippo, Russo, Francesco Paolo, Vegetti, Alberto, Balestro, Elisabetta, Pietrangelo, Antonello, Richeldi, Luca, Luppi, Fabrizio, Spagnolo, Paolo, Clini, Enrico, Cerri, Stefania, Cocconcelli, E, Tonelli, R, Abbati, G, Marchioni, A, Castaniere, I, Pelizzaro, F, Russo, F, Vegetti, A, Balestro, E, Pietrangelo, A, Richeldi, L, Luppi, F, Spagnolo, P, Clini, E, Cerri, S, Cocconcelli, Elisabetta, Tonelli, Roberto, Abbati, Gianluca, Marchioni, Alessandro, Castaniere, Ivana, Pelizzaro, Filippo, Russo, Francesco Paolo, Vegetti, Alberto, Balestro, Elisabetta, Pietrangelo, Antonello, Richeldi, Luca, Luppi, Fabrizio, Spagnolo, Paolo, Clini, Enrico, and Cerri, Stefania

Abstract

Data on the presence of subclinical fibrosis across multiple organs in patients with idiopathic lung fibrosis (IPF) are lacking. Our study aimed at investigating through hepatic transient elastography (HTE) the prevalence and clinical impact of subclinical liver fibrosis in a cohort of patients with IPF. Patients referred to the Centre for Rare Lung Disease of the University Hospital of Modena (Italy) from March 2012 to February 2013 with established diagnosis of IPF and without a documented history of liver diseases were consecutively enrolled and underwent HTE. Based on hepatic stiffness status as assessed through METAVIR score patients were categorized as "with liver fibrosis" (corresponding to a METAVIR score of F1-F4) and "without liver fibrosis" (METAVIR F0). Potential predictors of liver fibrosis were investigated through logistic regression model among clinical and serological variables. The overall survival (OS) was assessed according to liver fibrosis and multivariate Cox regression analysis was used to identify independent predictors. In 13 out of 37 patients (35%) with IPF, a certain degree of liver fibrosis was documented. No correlation was found between liver stiffness and clinical-functional parameters. OS was lower in patients 'with liver fibrosis' than in patients 'without liver fibrosis' (median months 33 [23-55] vs. 63 [26-94], p = 0.038). Patients 'with liver fibrosis' presented a higher risk of death at seven years as compared to patients 'without liver fibrosis' (HR = 2.6, 95% CI [1.003-6.7], p = 0.049). Higher level of AST to platelet ratio index (APRI) was an independent predictor of survival (HR = 4.52 95% CI [1.3-15.6], p = 0.02). In our cohort, more than one-third of IPF patients had concomitant subclinical liver fibrosis that negatively affected OS. These preliminary claims further investigation aimed at clarifying the mechanisms beyond multiorgan fibrosis and its clinical implication in patients with IPF.

Published
2021

18. 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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19. Subclinical liver fibrosis in patients with idiopathic pulmonary fibrosis

Author

Cocconcelli, E., Tonelli, R., Abbati, G., Marchioni, A., Castaniere, I., Pelizzaro, F., Russo, F. P., Vegetti, A., Balestro, E., Pietrangelo, A., Richeldi, Luca, Luppi, F., Spagnolo, P., Clini, E., Cerri, S., Richeldi L. (ORCID:0000-0001-8594-1448), Cocconcelli, E., Tonelli, R., Abbati, G., Marchioni, A., Castaniere, I., Pelizzaro, F., Russo, F. P., Vegetti, A., Balestro, E., Pietrangelo, A., Richeldi, Luca, Luppi, F., Spagnolo, P., Clini, E., Cerri, S., and Richeldi L. (ORCID:0000-0001-8594-1448)

Abstract

Data on the presence of subclinical fibrosis across multiple organs in patients with idiopathic lung fibrosis (IPF) are lacking. Our study aimed at investigating through hepatic transient elastography (HTE) the prevalence and clinical impact of subclinical liver fibrosis in a cohort of patients with IPF. Patients referred to the Centre for Rare Lung Disease of the University Hospital of Modena (Italy) from March 2012 to February 2013 with established diagnosis of IPF and without a documented history of liver diseases were consecutively enrolled and underwent HTE. Based on hepatic stiffness status as assessed through METAVIR score patients were categorized as “with liver fibrosis” (corresponding to a METAVIR score of F1–F4) and “without liver fibrosis” (METAVIR F0). Potential predictors of liver fibrosis were investigated through logistic regression model among clinical and serological variables. The overall survival (OS) was assessed according to liver fibrosis and multivariate Cox regression analysis was used to identify independent predictors. In 13 out of 37 patients (35%) with IPF, a certain degree of liver fibrosis was documented. No correlation was found between liver stiffness and clinical–functional parameters. OS was lower in patients ‘with liver fibrosis’ than in patients ‘without liver fibrosis’ (median months 33 [23–55] vs. 63 [26–94], p = 0.038). Patients ‘with liver fibrosis’ presented a higher risk of death at seven years as compared to patients ‘without liver fibrosis’ (HR = 2.6, 95% CI [1.003–6.7], p = 0.049). Higher level of AST to platelet ratio index (APRI) was an independent predictor of survival (HR = 4.52 95% CI [1.3–15.6], p = 0.02). In our cohort, more than one-third of IPF patients had concomitant subclinical liver fibrosis that negatively affected OS. These preliminary claims further investigation aimed at clarifying the mechanisms beyond multiorgan fibrosis and its clinical implication in patients with IPF.

Published
2020

20. Gaps in care of patients living with pulmonary fibrosis

Author

Moor, C.C. (Karen), Wijsenbeek-Lourens, M.S. (Marlies), Balestro, E. (Elisabetta), Biondini, D. (Davide), Bondue, B. (Benjamin), Cottin, V. (Vincent), Kreuter, M. (Michael), Bonella, F. (Francesco), Moor, C.C. (Karen), Wijsenbeek-Lourens, M.S. (Marlies), Balestro, E. (Elisabetta), Biondini, D. (Davide), Bondue, B. (Benjamin), Cottin, V. (Vincent), Kreuter, M. (Michael), and Bonella, F. (Francesco)

Abstract

Introduction: Pulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey. Methods: Two online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries. Results: The surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptomcentred management, adequate information, trial information and increasing awareness of PF/IPF is required. Conclusions: The surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience.

Published
2019
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22. Pretreatment rate of decay in forced vital capacity predicts long-term response to pirfenidone in patients with idiopathic pulmonary fibrosis

Author

Biondini, D, Balestro, E, Lacedonia, D, Cerri, S, Milaneschi, R, Luppi, F, Cocconcelli, E, Bazzan, E, Clini, E, Foschino Barbaro, M, Gregori, D, Cosio, M, Saetta, M, Spagnolo, P, Foschino Barbaro, MP, Cosio, MG, Biondini, D, Balestro, E, Lacedonia, D, Cerri, S, Milaneschi, R, Luppi, F, Cocconcelli, E, Bazzan, E, Clini, E, Foschino Barbaro, M, Gregori, D, Cosio, M, Saetta, M, Spagnolo, P, Foschino Barbaro, MP, and Cosio, MG

Abstract

Pirfenidone reduces functional decline in patients with Idiopathic Pulmonary Fibrosis (IPF). However, response to treatment is highly heterogeneous. We sought to evaluate whether response to pirfenidone is influenced by the pretreatment rate of forced vital capacity (FVC) decline. Fifty-six IPF patients were categorized as rapid (RP) or slow progressors (SP) based on whether their FVC decline in the year preceding pirfenidone treatment was > or ≤ 10% predicted. Following pirfenidone treatment patients were followed-up every 6 months and up to 24 months. In the entire population, pirfenidone reduced significantly FVC decline from 231 to 49 ml/year at 6 months (T6) (p = 0.003) and this effect was maintained at the 12-, 18- and 24-month time points (p value for trend n.s.). In RP, the reduction of FVC decline was evident at 6 months (36 vs 706 ml/year pretreatment; p = 0.002) and maintained, though to a lesser degree, at 12 (106 ml/year), 18 (176 ml/year) and 24 months (162 ml/year; p value for trend n.s). Among SP, the reduction in FVC decline was not significant at any of the time points analyzed. In conclusion, pirfenidone reduces FVC decline in IPF patients. However, its beneficial effect is more pronounced in patients with rapidly progressive disease.

Published
2018

23. A Real-Life Multicenter National Study on Nintedanib in Severe Idiopathic Pulmonary Fibrosis

Author

Harari, S, Caminati, A, Poletti, V, Confalonieri, M, Gasparini, S, Lacedonia, D, Luppi, F, Pesci, A, Sebastiani, A, Spagnolo, P, Vancheri, C, Balestro, E, Bonifazi, M, Cerri, S, De Giacomi, F, Della Porta, R, Foschino Barbaro, M, Fui, A, Pasquinelli, P, Rosso, R, Tomassetti, S, Specchia, C, Rottoli, P, Harari, Sergio, Caminati, Antonella, Poletti, Venerino, Confalonieri, Marco, Gasparini, Stefano, Lacedonia, Donato, Luppi, Fabrizio, Pesci, Alberto, Sebastiani, Alfredo, Spagnolo, Paolo, Vancheri, Carlo, Balestro, Elisabetta, Bonifazi, Martina, Cerri, Stefania, De Giacomi, Federica, Della Porta, Rossana, Foschino Barbaro, Maria Pia, Fui, Annalisa, Pasquinelli, Patrizio, Rosso, Roberta, Tomassetti, Sara, Specchia, Claudia, Rottoli, Paola, Harari, S, Caminati, A, Poletti, V, Confalonieri, M, Gasparini, S, Lacedonia, D, Luppi, F, Pesci, A, Sebastiani, A, Spagnolo, P, Vancheri, C, Balestro, E, Bonifazi, M, Cerri, S, De Giacomi, F, Della Porta, R, Foschino Barbaro, M, Fui, A, Pasquinelli, P, Rosso, R, Tomassetti, S, Specchia, C, Rottoli, P, Harari, Sergio, Caminati, Antonella, Poletti, Venerino, Confalonieri, Marco, Gasparini, Stefano, Lacedonia, Donato, Luppi, Fabrizio, Pesci, Alberto, Sebastiani, Alfredo, Spagnolo, Paolo, Vancheri, Carlo, Balestro, Elisabetta, Bonifazi, Martina, Cerri, Stefania, De Giacomi, Federica, Della Porta, Rossana, Foschino Barbaro, Maria Pia, Fui, Annalisa, Pasquinelli, Patrizio, Rosso, Roberta, Tomassetti, Sara, Specchia, Claudia, and Rottoli, Paola

Abstract

Background: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. Objectives: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. Methods: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib. The aim of the study was to compare the decline in lung function before and after treatment. Patient survival after 6 months of therapy with nintedanib was assessed. Results: Forty-one patients with a forced vital capacity (FVC) ≤50% and/or a diffusing capacity of the lung for carbon monoxide (DLCO) ≤35% predicted at the start of nintedanib treatment were enrolled. At the 6-month follow-up, the decline of DLCO (both absolute and % predicted) was significantly reduced compared to the pretreatment period (absolute DLCO at the -6-month, T0, and +6-month time points (5.48, 4.50, and 5.03 mmol/min/kPa, respectively, p = 0.03; DLCO% predicted was 32.73, 26.54, and 29.23%, respectively, p = 0.04). No significant beneficial effect was observed in the other functional parameters analyzed. The 1-year survival in this population was 79%, calculated from month 6 of therapy with nintedanib. Conclusions: This nationwide multicenter experience in patients with severe IPF shows that nintedanib slows down the rate of decline of absolute and % predicted DLCO but does not have significant impact on FVC or other lung parameters.

Published
2018

24. Selection of Candidates for Lung Transplantation: The First Italian Consensus Statement

Author

Nosotti, M., primary, Dell'Amore, A., additional, Diso, D., additional, Oggionni, T., additional, Aliberti, S., additional, Balestro, E., additional, Bertani, A., additional, Boffini, M., additional, Lucianetti, A., additional, Luzzi, L., additional, Paone, G., additional, Parigi, P., additional, Pellegrini, C., additional, Rocca, A., additional, Rottoli, P., additional, Santambrogio, L., additional, Schiavon, M., additional, Solidoro, P., additional, Vitulo, P., additional, and Tarsia, P., additional

Published
2017
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25. Antibiotic treatment of severe exacerbations of chronic obstructive pulmonary disease with procalcitonin: a randomized noninferiority trial

Author

Verduri, A, Luppi, F, D'Amico, R, Balduzzi, S, Vicini, R, Liverani, A, Ruggieri, V, Plebani, M, Barbaro, Mp, Spanevello, A, Canonica, Gw, Papi, A, Fabbri, Lm, Beghè, B, FARM58J2XH Study Group: Fabbri LM, Beghé, B, Franco, F, De Carlo MR, Confalonieri, M, Milani, G, Pozzi, E, Luisetti, M, Cerveri, I, Niniano, R, Marsico, S, Calabrese, C, Olivieri, D, Tzani, P, Torre, O, Braido, F, Salerno, F, Carone, M, Zucchi, L, Menzella, F, Castagnetti, C, Paggiaro, Pl, Vagaggini, B, Costa, F, Contoli, M, Marku, B, Cagnazzo, Mg, Crimi, Nunzio, Mastruzzo, C, Ciccarelli, M, Calabro, S, Balestro, E, Rossi, A, Donazzan, G, Bonazza, L, Zuin, R, Pistolesi, M, and Bigazzi, F.

Subjects
Sepsis, C-Reactive Protein, Serum procalcitonin
Published
2015

26. L’attività dei Centri Antifumo italiani tra problematiche e aree da potenziare: i risultati di un’indagine svolta attraverso un questionario on-line

Author

Alessandra Di Pucchio, Roberta, Pacifici, Simona, Pichini, Antonella, Bacosi, Simonetta Di Carlo, Rita Di Giovannandrea, Patrizia, Gori, (ASL Brescia, Enrica Pizzi e Gruppo Servizi Territoriali per la cessazione dal fumo di tabacco: Abrami M. A., Leno, -BS), Agus, A., (ASS2 Isontina, Nicolazzi L., Gorizia), Ameglio, M., (AUSL 7, Cincinelli B., Zona Alta Val d'Elsa, Siena), Badii, F., (ASL 2 Savonese, Grossi S., Savona), Baraldo M. (OU S. Maria della Misericordia, Udine), Barbano, G., (AULSS 9 - Ospedale Cà Foncello, Bazzerla G., Treviso), Bassini M., Giampaolo R. (AUSL di Modena -Distretto di Castelfranco Emilia -MO), Beato, E., (Dipartimento Dipendenze, Prestipino A., ASL di Bergamo), Bergozza L., Rossetto L. (ULSS 4 Thiene -VI), Bertoletti R. (AO Valtellina e Valchiavenna, Sondalo), Biasin, C., (ULSS 21, Manzato E., Zevio, -VR), Bigarelli M. (AUSL Modena Area Nord, Carpi -MO), (ASL della Provincia di Mantova), Bini L., Boffi, R., (Fondazione IRCCS Istituto Nazionale dei Tumori, Pozzi P., Milano), Brancaccio, L., Monaldi', Guarino C. (AO 'V., Napoli), Cacaci, C., (ASUR Marche - Area Vasta 5, Apolloni P., San Benedetto del Tronto -AP), Calcagni, A. M., (Area Vasta 4 - Regione Marche, Mecozzi L., Giorgio -FM), Porto S., Calzolari, U., (ASL di Bergamo, Rovetta M., Lovere, -BG), Campiotti A. (ASL Milano 1, Magenta -MI), Canneti, E., Saccocci, M., Misericordia, Vichi S. (USL 9 P. O., Grosseto), Canzian G., Russo A. (ASS 3 Alto Friuli), Cao A. (ASL 5, Oristano), Carnevale E. (ASL Napoli 1 Centro - PS 'S. Maria di Loreto Crispi', Napoli), Carone, M., Maugeri, Sarno N. (Fondazione S., Cassano delle Murge -BA), Carrozzi, L., Pistelli, F., Cavalleri, C., (ASL TO3, Pini D., Beinasco, -TO), Cerrato P. (ASL TO2, Torino), Cestaro C., Repetto T. (AUSL Valle d'Aosta), Ciarfeo Purich R., Vegliach A. (ASS 1 Triestina), (Ausl di Imola), Cifiello S., Colombari, P., (ASL VC, Conti P., Vercelli), Conterio, P., (Asl TO1 Ospedale Martini, Gallo E., Torino), Cordioli, E., ULSS 22 Bussolengo, Melchiori S. (Az., Verona), Corti M. (ASL provincia Bergamo, Treviglio -BG), Costantino A. (AO Pugliese Ciaccio, Catanzaro), Cuccagna, P., (Asl TO3, Potosnjack A., Perosa Argentina -TO), Cutrì, R., (Azienda 10 Lungarno Santa Rosa, Barbanti A., Firenze), D’Urso, B., San Giovanni di Dio Ruggi D’Aragona', Amendola D. (AOU 'OO. RR., Salerno), Da Ros D. (ULSS 16, Padova), (IRCCS Ospedale, D'Alessandro V., Dalla Pietra G., Pavani V. (Az. ULSS 18 Rovigo), De Benedetto, F., Annunziata, Manigrasso M. R. (ASL Chieti PO Clinicizzato S. S., Chieti Scalo -CH), De Donno G. (AO Carlo Poma, Mantova), De Lozzo L., Facchini F. (PO di Vittorio Veneto -TV), De Marchi C. (ASL BI, Cossato -BI), (Sert Lamezia Terme), De Siena A., (ASP Reggio Calabria), De Stefano C., Del Donno, M., Rummo', Romano A. (AO 'G., Benevento), Delfini M., Galati A. (P. O. Villa Betania RM E), Di Dio, S., (ASP 3 Catania, Franco R., Giarre, -CT), Dominici F., De Rose V. (ASL di Viterbo), Donateo L., Gerardi R. (ASL Lecce), Fagni, F., (AUSL 3 di Pistoia, Pellegrini V., Pescia, -PT), Foschino Barbaro, M. P., (AOU Policlinico- Ospedale D'Avanzo, Ruggieri C., Foggia), Fulgione, C., Gennaro, Mallardo M. (ASL NA 1 O S., Fusconi, E., (ASL RMC Distretto 9, Del Brocco D., Roma), Galletti, F., (AOU Federico II, De Palma D., Grammatico P. (ASL Roma D, Roma), Grandelis C. (APSS, Borgo Valsugana -TN), Groppi C. (ASL 3, Pistoia), Iacoacci C., Barbieri F. (INRCA di Ancona), Ianniello, F., (AUSL 8, Passeri A., Montevarchi, -AR), Janiri L. (Università Cattolica Sacro Cuore, Roma), Kashanpour, H., (ASS 4, Zanon D., Udine), (PO Sud-Ospedale di Gaeta -LT), Kugler E., Lauro G., D'Amore A. (ASL Caserta), Lavacchini, G., (ASL 10 FI, Grati D., Zona, Mugello, Lorenzo -FI), Borgo S., Leonardi, C., (ASL RMC, Fierro G., Leonetti A. (ASL CN1, Mondovì -CN), Lugoboni, F., (AO Universitaria, Casari R., (ARNAS Ospedale Civico di Palermo), Madonia G., Magnelli F., Rota A. G. (ASP di Cosenza), Maiella, D., (ASL BN1, Izzo A., Manfredi A., Gai P. (ASL 4 di Prato), Mantovani A., Finessi A. (Az. Ulss 19 Adria -RO), Mantovani R., Viviani U. (AUSL di Ferrara PO di Copparo -FE), (ASP Trapani PO Mazara del Vallo -TP), Marino G., (ASL Sassari), Marras T., (ASL Ferrara), Marsili R., (ULSS 13 Dolo -VE), Melica V. E., Messere C. (ASL NA2 Nord, Napoli), Monti M. (Istituto Ortopedico Rizzoli, Bologna), Mussoni C., Cavicchi C. (Az. USL Bologna), Natoli, A., (ASS 6, Furlan N., Pordenone), Pamerani B., Porciatti C. (AUSL 10 Firenze), Panzanella C. (ASL Napoli 1 Centro – Osp 'San Giovanni Bosco', Napoli), Paolin C., Cannarsa F. (Az. Ulss 7 Pieve di Soligo -TV), Parillo T., Hazra C. (ASL di Como), Pasinato M. G. (Ulss 8 Asolo, Castelfranco Veneto -TV), (ASL 1 Distretto di Alghero -SS), Peana G., Pellegrini, L., Gios, L., (Distretto Centro Sud, Herzog S., Rovereto, -TN), Perillo, A., (ASL Na 3 Sud, Ambrosino M., Pomigliano d'Arco -NA), (Azienda Sanitaria di Matera), Perna M. T., Pezzuto, A., Andrea- Università Sapienza, Mariotta S. (AO S., Piancastelli, G., Ricci, C., Fabbri, C., USL Ravenna, Fava P. (Az., Sedi di Ravenna, Bagnacavallo, Faenza, -RA), Pieralli, D., (AUSL 8 Zona Aretina, Ferrarotto E., Arezzo), Pistelli, R., (Università Cattolica, Villani A., Complesso Integrato Columbus, Polo, M. F., (AOU, Manca S., Sassari), Polosa, R., (AOU, Caponnetto P., Università di Catania), Principe, R. Clementi F., Camillo-Forlanini, Dragani L. (AO S., Pulerà N. (AUSL 6, Livorno), Puoti F. (ASL Na1, Napoli), Residori M. (AULSS 22 Regione Veneto, Villafranca -VR), Romagnolo A. (ASL AT, Asti), Romano, A., Russo, G., (ASL TO 4 Ivrea, Arimatea S., Chivasso, Ciriè, -TO), Romano F., Scarlato M. I. (AO di Cosenza), Sacchi, G., Alletto', Magro F. (ASL 1-Poliambulatorio 'U., Agrigento), Saetta, M., Forza, G., (PO di Padova), Balestro E., Serafini, A., (Ospedale Civile, Bertora G., Imperia), Siracusano, L., (Istituto Clinico Humanitas, Autuori M., Rozzano, -MI), Spada, E., Maugeri, Balestroni G. (Fondazione S., Veruno, -NO), Staccioli M. C., Rossi B. (Ausl Rimini), Stefano, V., (ASL Provincia di Varese, Gaggini R., Arcisate, -VA), Subiaco, S., (ASUR Marche, Cimarelli M. E., Area Vasta, 2 Jesi -AN), Tartarotti I. (APSS, Pergine -TN), Tavanti, G., (AUSL 10, Paparini L., Antella, -FI), Tinghino B. (ASL Provincia di Monza e Brianza, Monza), (ASP N9 Trapani), Trapani V., Triani A., Senoner A. (AS Merano e AS Bolzano -BZ), Valeri W. (Area Vasta 2 Ancona – ASUR Marche, Ancona), (ASL di Lodi), Varango C., Varese M. (ASL 1, Regione Toscana), Vidal, V., (ASS 5, Mustacchi A., Palmanova, -UD), Viola, S., Salvini, Tomasich A. (A. O., Garbagnate, -MI), (AO Istituti Ospitalieri di Cremona -CR), Virzì G., Vito, A., Parrella, R. (AORN Ospedali dei Colli Monaldi-Cotugno-C. T. O., (AUSL di Bologna), Zagà V., and (ULSS 5, Zini G.

Subjects
Centri Antifumo, Indagine on-line, Cessazione dal fumo di tabacco, Fumo di tabacco
Published
2013

37. Noneosinophilic asthma in children: relation with airway remodelling

Author

Baraldo, S., primary, Turato, G., additional, Bazzan, E., additional, Ballarin, A., additional, Damin, M., additional, Balestro, E., additional, Lokar Oliani, K., additional, Calabrese, F., additional, Maestrelli, P., additional, Snijders, D., additional, Barbato, A., additional, and Saetta, M., additional

Published
2011
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46. A novel insight into adaptive immunity in chronic obstructive pulmonary disease: B cell activating factor belonging to the tumor necrosis factor family.

Author

Polverino F, Baraldo S, Bazzan E, Agostini S, Turato G, Lunardi F, Balestro E, Damin M, Papi A, Maestrelli P, Calabrese F, and Saetta M

Abstract

Rationale: Chronic obstructive pulmonary disease (COPD) is a disorder characterized by an abnormal inflammatory response that persists even after smoking cessation, yet the underlying mechanisms are not fully understood. Objectives: To investigate the expression of B-cell activating factor of tumor necrosis factor family (BAFF), a crucial mediator in the crosstalk between innate and adaptive immune responses, in patients with COPD and to explore its correlation with disease severity. Methods: Using immunohistochemistry, expression of BAFF was examined in lung specimens from 21 smokers with COPD (FEV(1) = 57 ± 5% predicted), 14 control smokers (FEV(1) = 99 ± 2% predicted) and 8 nonsmokers (FEV(1) = 104 ± 4% predicted). BAFF was quantified in alveolar macrophages and alveolar walls, in bronchiolar and parenchymal lymphoid follicles, and in peripheral airways and pulmonary arterioles. Measurements and Main Results: In alveolar macrophages and parenchymal lymphoid follicles, BAFF expression was increased in smokers with COPD compared with control smokers and nonsmokers (P < 0.05 for all comparisons). In both compartments, BAFF was also up-regulated in control smokers as compared with nonsmokers (P = 0.03 and P = 0.01). Moreover, BAFF was overexpressed in bronchiolar lymphoid follicles, alveolar walls, peripheral airways, and pulmonary arterioles from smokers with COPD compared with nonsmokers (P < 0.05 for all). Among patients with COPD, BAFF(+) macrophages were inversely related to FEV(1) (P = 0.03, Spearman's rho [r(S)] = -0.48), FEV(1)/FVC (P = 0.02, r(S) = -0.50), and Pa(O(2)) values (P = 0.01, r(S) = -0.55). Conclusions: This study demonstrated overexpression of BAFF in peripheral lung of patients with COPD, mainly in alveolar macrophages and lymphoid follicles. Moreover, BAFF expression was correlated to the degree of lung function impairment and hypoxia, suggesting that it may have a possible impact on disease severity. [ABSTRACT FROM AUTHOR]

Published
2010
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47. Viral infections in lung transplant recipients: Devils or trolls?

Author

Solidoro, P., Balestro, E., and Massimo Boffini

Subjects
Lung transplantation, Diagnosis, Virus disease
Abstract

Lung transplantation is a therapeutic option for end stage lung diseases. One of the most important topics in transplant management is the role of viral infections in chronic lung allograft dysfunction (CLAD) and in particular in acute rejection (AR). This review arise from a recent study BY Brideaux et al. that offers the opportunity to investigate deeply the incidence, risk factors, symptomatology and clinical outcome of respiratory viral infections. Although most respiratory viral infections cause self-limited upper respiratory diseases, lung transplant recipients (LTRs) are particularly prone to develop complications. The absence of symptoms is a pivotal problem in managing these patients as it can depend on absence of active replication or on the effect of immunosuppressive regimen. In one word viruses can be just passengers or aggressive drivers in a facilitated environment, and the potential damage is completely different, as the management. PCR samplings give us an idea of the presence but not the certainty of the activity of viruses, and this is another common problem in reading data. In Herpes Virus infections this problem can be overtaken by studying biological samples and immune response, balancing the presence (PCR) and the activity (shell vial) of viruses with specific immune response (elispot). In fact viral presence doesn't mean activity and activity doesn't mean pathology in case of competent immune response. All these data can be matched in every single patient and managed by a tailored approach, either monitoring or treating.

48. Telomeropathies: an emerging spectrum of disorders with important implications for patients with interstitial lung disease

Author

Stella, G. M., Balestro, E., Donato Lacedonia, and Baraldo, S.

Subjects
Humans, Chromosome Disorders, Telomere, Lung Diseases, Interstitial
Abstract

Growing evidence demonstrates that a number of clinical disorders may be related to genetic defects in telomeres replication and extensions. Overall, these syndromes are referred to as telomeropathies or telomeres disorders or syndromes; they are increasingly being identified. In adulthood, idiopathic pulmonary fibrosis (IPF) is the most common symptom of telomeropathy. IPF is a progressive and fatal disease characterized by scarring of the lungs that thickens the interstitium ultimately leading to irreversible respiratory failure. Starting from this basis, the present review analyzes and discusses the findings of a relevant paper by Gautam George and colleagues from the Division of Pulmonary and Critical Care Medicine at Brigham and Women's Hospital and Harvard Medical School in Boston (USA) recently appeared on the prestigious journal CHEST. In a cohort of patients addressed to lung transplantation, authors were able to demonstrate that subclinical bone marrow and liver abnormalities can be seen in patients with interstitial lung disease (ILD) and short telomeres, in some cases in the absence of clinically significant abnormalities in peripheral blood count and liver function tests. This observation sustains the rationale for further studies aimed to validate telomere length testing as a useful parameter as part of the evaluation for transplant candidacy. A deeper clarification of the complex link between IPF and short telomeres and telomeropathies is required for a new ILD classification, aimed to a fullpersonalized approach to the disease.

49. Undifferentiated connective tissue disease presenting with prevalent interstitial lung disease: Case report and review of literature

Author

Sfriso Paolo, Polverosi Roberta, Cozzi Franco, Nordio Beatrice, Balestro Elisabetta, Lunardi Francesca, Braccioni Fausto, and Calabrese Fiorella

Subjects
Pathology, RB1-214
Abstract

Abstract Undifferentiated connective tissue diseases (UCTDs) are clinical entities characterised by signs and symptoms suggestive of a systemic autoimmune disease, which do not fulfil the diagnostic criteria for a defined connective tissue disease. Lung involvement can complicate the course and management of the disease, often determining a worse outcome. Respiratory dysfunction as the first clinical manifestation has seldom been reported. We describe a case of a female patient who developed significant respiratory dysfunction as the principal clinical sign. Video-assisted thoracoscopy was performed and a histological pattern of nonspecific interstitial pneumonia (NSIP) was found. A pathological diagnosis suggested careful follow-up with extensive immunological screening which then detected Raynaud's phenomenon and positivity of antinuclear antibodies. After a multidisciplinary discussion (pneumologist, radiologist, pathologist and rheumatologist) a final diagnosis of NSIP associated with UCTD was made. The diagnosis of UCTD should be considered when NSIP is diagnosed even in cases with evident first clinical manifestations of severe respiratory dysfunction. A multidisciplinary approach in the field of interstitial lung disease with NSIP, also including rheumatologic expertise, is fundamental to achieve a prompt and correct diagnosis.

Published
2011
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50. Pretreatment rate of decay in forced vital capacity predicts long-term response to pirfenidone in patients with idiopathic pulmonary fibrosis

Author

Fabrizio Luppi, Marina Saetta, Elisabetta Balestro, Donato Lacedonia, Manuel G. Cosio, Elisabetta Cocconcelli, Stefania Cerri, Maria Pia Foschino Barbaro, Erica Bazzan, Dario Gregori, Paolo Spagnolo, Rosanna Milaneschi, Davide Biondini, Enrico Clini, Biondini, D, Balestro, E, Lacedonia, D, Cerri, S, Milaneschi, R, Luppi, F, Cocconcelli, E, Bazzan, E, Clini, E, Foschino Barbaro, M, Gregori, D, Cosio, M, Saetta, M, and Spagnolo, P

Subjects
Adult, Male, medicine.medical_specialty, Vital capacity, Pyridones, Vital Capacity, lcsh:Medicine, Rate of decay, Gastroenterology, Article, 03 medical and health sciences, Idiopathic pulmonary fibrosis, FEV1/FVC ratio, 0302 clinical medicine, forced vital capacity, Internal medicine, idiopathic pulmonary fibrosis, pirfenidone, forced vital capacity, medicine, Humans, In patient, 030212 general & internal medicine, Longitudinal Studies, Prospective Studies, lcsh:Science, Aged, Multidisciplinary, idiopathic pulmonary fibrosi, business.industry, Anti-Inflammatory Agents, Non-Steroidal, lcsh:R, Pirfenidone, Middle Aged, respiratory system, medicine.disease, Idiopathic Pulmonary Fibrosis, respiratory tract diseases, Long term response, Treatment Outcome, 030228 respiratory system, Disease Progression, Female, lcsh:Q, pirfenidone, business, Progressive disease, medicine.drug
Abstract

Pirfenidone reduces functional decline in patients with Idiopathic Pulmonary Fibrosis (IPF). However, response to treatment is highly heterogeneous. We sought to evaluate whether response to pirfenidone is influenced by the pretreatment rate of forced vital capacity (FVC) decline. Fifty-six IPF patients were categorized as rapid (RP) or slow progressors (SP) based on whether their FVC decline in the year preceding pirfenidone treatment was > or ≤ 10% predicted. Following pirfenidone treatment patients were followed-up every 6 months and up to 24 months. In the entire population, pirfenidone reduced significantly FVC decline from 231 to 49 ml/year at 6 months (T6) (p = 0.003) and this effect was maintained at the 12-, 18- and 24-month time points (p value for trend n.s.). In RP, the reduction of FVC decline was evident at 6 months (36 vs 706 ml/year pretreatment; p = 0.002) and maintained, though to a lesser degree, at 12 (106 ml/year), 18 (176 ml/year) and 24 months (162 ml/year; p value for trend n.s). Among SP, the reduction in FVC decline was not significant at any of the time points analyzed. In conclusion, pirfenidone reduces FVC decline in IPF patients. However, its beneficial effect is more pronounced in patients with rapidly progressive disease.

Published
2018

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