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1. Idiopathic Pulmonary Fibrosis Is Associated with Common Genetic Variants and Limited Rare Variants.

Author

Peljto, Anna L, Blumhagen, Rachel Z, Walts, Avram D, Cardwell, Jonathan, Powers, Julia, Corte, Tamera J, Dickinson, Joanne L, Glaspole, Ian, Moodley, Yuben P, Vasakova, Martina Koziar, Bendstrup, Elisabeth, Davidsen, Jesper R, Borie, Raphael, Crestani, Bruno, Dieude, Philippe, Bonella, Francesco, Costabel, Ulrich, Gudmundsson, Gunnar, Donnelly, Seamas C, Egan, Jim, Henry, Michael T, Keane, Michael P, Kennedy, Marcus P, McCarthy, Cormac, McElroy, Aoife N, Olaniyi, Joshua A, O'Reilly, Katherine MA, Richeldi, Luca, Leone, Paolo M, Poletti, Venerino, Puppo, Francesco, Tomassetti, Sara, Luzzi, Valentina, Kokturk, Nurdan, Mogulkoc, Nesrin, Fiddler, Christine A, Hirani, Nikhil, Jenkins, R Gisli, Maher, Toby M, Molyneaux, Philip L, Parfrey, Helen, Braybrooke, Rebecca, Blackwell, Timothy S, Jackson, Peter D, Nathan, Steven D, Porteous, Mary K, Brown, Kevin K, Christie, Jason D, Collard, Harold R, Eickelberg, Oliver, Foster, Elena E, Gibson, Kevin F, Glassberg, Marilyn, Kass, Daniel J, Kropski, Jonathan A, Lederer, David, Linderholm, Angela L, Loyd, Jim, Mathai, Susan K, Montesi, Sydney B, Noth, Imre, Oldham, Justin M, Palmisciano, Amy J, Reichner, Cristina A, Rojas, Mauricio, Roman, Jesse, Schluger, Neil, Shea, Barry S, Swigris, Jeffrey J, Wolters, Paul J, Zhang, Yingze, Prele, Cecilia MA, Enghelmayer, Juan I, Otaola, Maria, Ryerson, Christopher J, Salinas, Mauricio, Sterclova, Martina, Gebremariam, Tewodros H, Myllärniemi, Marjukka, Carbone, Roberto G, Furusawa, Haruhiko, Hirose, Masaki, Inoue, Yoshikazu, Miyazaki, Yasunari, Ohta, Ken, Ohta, Shin, Okamoto, Tsukasa, Kim, Dong Soon, Pardo, Annie, Selman, Moises, Aranda, Alvaro U, Park, Moo Suk, Park, Jong Sun, Song, Jin Woo, Molina-Molina, Maria, Planas-Cerezales, Lurdes, Westergren-Thorsson, Gunilla, Smith, Albert V, Manichaikul, Ani W, and Kim, John S

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Autoimmune Disease, Lung, Rare Diseases, Genetics, Human Genome, Prevention, Precision Medicine, 2.1 Biological and endogenous factors, Good Health and Well Being, Humans, Idiopathic Pulmonary Fibrosis, Whole Genome Sequencing, Exome, whole-genome sequencing, interstitial lung disease, TOPMed, genetic association studies, telomerase, Medical and Health Sciences, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

Rationale: Idiopathic pulmonary fibrosis (IPF) is a rare, irreversible, and progressive disease of the lungs. Common genetic variants, in addition to nongenetic factors, have been consistently associated with IPF. Rare variants identified by candidate gene, family-based, and exome studies have also been reported to associate with IPF. However, the extent to which rare variants, genome-wide, may contribute to the risk of IPF remains unknown. Objectives: We used whole-genome sequencing to investigate the role of rare variants, genome-wide, on IPF risk. Methods: As part of the Trans-Omics for Precision Medicine Program, we sequenced 2,180 cases of IPF. Association testing focused on the aggregated effect of rare variants (minor allele frequency ⩽0.01) within genes or regions. We also identified individual rare variants that are influential within genes and estimated the heritability of IPF on the basis of rare and common variants. Measurements and Main Results: Rare variants in both TERT and RTEL1 were significantly associated with IPF. A single rare variant in each of the TERT and RTEL1 genes was found to consistently influence the aggregated test statistics. There was no significant evidence of association with other previously reported rare variants. The SNP heritability of IPF was estimated to be 32% (SE = 3%). Conclusions: Rare variants within the TERT and RTEL1 genes and well-established common variants have the largest contribution to IPF risk overall. Efforts in risk profiling or the development of therapies for IPF that focus on TERT, RTEL1, common variants, and environmental risk factors are likely to have the largest impact on this complex disease.

Published
2023

3. Profiling severe asthma: Any relevance for age? An analysis from Severe Asthma Network Italy (SANI) cohort

Author

Brussino, Luisa, Calabrese, Cecilia, Camiciottoli, Gianna, Carpagnano, Giovanna E., Centanni, Stefano, Corsico, Angelo G., Costantino, Maria T., Crimi, Claudia, D'adda, Alice, D'alo, Simona, D'amato, Maria, Del Giacco, Stefano, Di Marco, Fabiano, Facciolongo, Nicola C., Latorre, Manuela, Nettis, Eustachio, Nucera, Eleonora, Passalacqua, Giovanni, Pelaia, Girolamo, Pini, Laura, Ricciardi, Luisa, Richeldi, Luca, Ridolo, Erminia, Santus, Pierachille, Scichilone, Nicola, Scioscia, Giulia, Spadaro, Giuseppe, Spanevello, Antonio, Tarsia, Paolo, Caminati, Marco, Marcon, Alessandro, Vaia, Rachele, Senna, Gianenrico, Maule, Matteo, Marchetti, Pierpaolo, Miotti, Jessica, Argentino, Giuseppe, Blasi, Francesco, Canonica, Giorgio W., Heffler, Enrico M., Paggiaro, Pierluigi, Vianello, Andrea, and Guarnieri, Gabriella

Published
2024
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4. High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study

Author

Anderson, Adam, Morgenthau, Adam, Gaser, Adrian, Vizel, Alexander, Speranskaya, Alexandra, Gerke, Alicia, Goksel, Altinisik, Undurraga, Alvaro, Sharma, Amita, Oh, Andrea, Leung, Ann, Larici, Anna Rita, Prasse, Antje, Mazzei, Antonietta, Morais, Antonio, Bhalla, Ashu, Del Rio, Belen, Jankharia, Bhavin, Elicker, Brett, Pereira, Carlos, Biegelman-Aubry, Catherine, White, Charles, Ravaglia, Claudia, Hsia, Connie, Schaefer-Prokop, Cornelia, Launay, David, Talwar, Deepak, Castillo, Diego, Patel, Divya, Israel-Biet, Dominique, Valeyre, Dominique, Britt, Edward James, Bargagli, Elena, Bendstrup, Elisabeth, Crouser, Elliott, Nossent, Esther, Shmelev, Eugeny, Porquera, Eva Carmona, Bonella, Francesco, Cohen-Aubart, Fleur, Jeny, Florence, Ferrara, Giovanni, Jin, Gong Yong, Robbie, Hasti, Prosch, Helmut, Sumikawa, Hiromitsu, Ling-Pei, Ho, Lee, Ho Yun, Strambu, Irina, Buendia-Roldan, Ivette, Grutters, Jan, Ryu, Jay, Swigris, Jeff, Miedema, Jelle, Goo, Jin Mo, Barnett, Joseph, Verschakelen, Johny, Goldin, Jonathan, Seo, Joon Beom, Enghelmayer, Juan, Mana, Juan, Behr, Juergen, Patterson, Karen, Antoniou, Katerina, Brown, Kevin, Fujimoto, Kiminori, Savale, Laurent, Maier, Lisa, Richeldi, Luca, Neto, Manuel Lessa Ribeiro, Humbert, Marc, Judson, Marc, Veltkamp, Marcel, Wilsher, Margaret, Molina, Maria, Otaola, Maria, Revel, Marie-Pierre, Silva, Mario, Drent, Marjolein, Schiebler, Mark, Wijsenbeek-Lourens, Marlies, Bonifazi, Martina, Remy-Jardin, Martine, Koslow, Matthew, Balter, Meyer, Kreuter, Michael, Thillai, Muhunthan, Hamzeh, Nabeel, Chaudhuri, Nazia, Mogulkoc, Nesrin, Todd, Nevins, Screaton, Nick, Goh, Nicole, Shah, Nirav, Obi, Ogugua, Shlobin, Oksana, Baranova, Olga, Bresser, Paul, Rottoli, Paula, Brillet, Pierre-Yves, Said-Hartley, Qonita, Borie, Raphael, Mostard, Remy, van Zyl-smit, Richard, Kairalla, Ronaldo, Egashira, Ryoko, Boussouar, Samia, Lee, Sang Min, Bhalla, Sanjeev, Tomassetti, Sara, Quadrelli, Silvia, Hart, Simon P, Danoff, Sonye, Rajan, Sujeet, Choi, Sun Mi, Copley, Susan, Kim, Tae Jung, Johkoh, Takeshi, Corte, Tamera, McLoud, Theresa, Wessendorf, Thomas, Alfaro, Tiago, Arai, Toru, Costabel, Ulrich, Vicens-Zygmunt, Vanesa, Poletti, Venerino, Cottin, Vincent, Vucinic, Violeta, Wuyts, Wim, van Es, Wouter, Jeong, Yeon Joo, Inoue, Yoshikazu, Desai, Sujal R, Sivarasan, Nishanth, Johannson, Kerri A, George, Peter M, Culver, Daniel A, Devaraj, Anand, Lynch, David A, Milne, David, Renzoni, Elisabetta, Nunes, Hilario, Sverzellati, Nicola, Spagnolo, Paolo, Baughman, Robert P, Yadav, Ruchi, Piciucchi, Sara, Walsh, Simon L F, Kouranos, Vasileios, and Wells, Athol U

Published
2024
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7. Detection and Early Referral of Patients With Interstitial Lung Abnormalities An Expert Survey Initiative

Author

Hunninghake, Gary M, Goldin, Jonathan G, Kadoch, Michael A, Kropski, Jonathan A, Rosas, Ivan O, Wells, Athol U, Yadav, Ruchi, Lazarus, Howard M, Abtin, Fereidoun G, Corte, Tamera J, de Andrade, Joao A, Johannson, Kerri A, Kolb, Martin R, Lynch, David A, Oldham, Justin M, Spagnolo, Paolo, Strek, Mary E, Tomassetti, Sara, Washko, George R, White, Eric S, Group, ILA Study, Abtin, Fereidoun, Antoniou, Katerina, Blackwell, Timothy, Brown, Kevin, Chung, Jonathan, Corte, Tamera, Crestani, Bruno, Crossno, Peter, Culver, Daniel, de Andrade, Joao, Deveraj, Anand, Flaherty, Kevin, Gudmundsson, Gunnar, Hatabu, Hiroto, Jacob, Joe, Johansson, Kerri, Kanne, Jeff, Kazerooni, Ella, Kolb, Martin, Lynch, David, Maher, Toby, Martinez, Fernando, Morais, Antonio, Nathan, Steven D, Noth, Imre, Oldham, Justin, Podolanczuk, Anna, Poletti, Venerino, Ravaglia, Claudia, Renzoni, Elizabetta, Richeldi, Luca, Rubin, Geoffrey, Ryerson, Chris, Sahoo, Debasis, Suh, Rob, Sverzellati, Nicola, Valeyre, Dominique, Walsh, Simon, and Washko, George

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Prevention, Biomedical Imaging, Lung, Clinical Research, 4.4 Population screening, Detection, screening and diagnosis, Respiratory, Disease Progression, Early Diagnosis, Female, Humans, Lung Diseases, Interstitial, Male, Pulmonologists, Radiologists, Referral and Consultation, Respiratory Function Tests, Surveys and Questionnaires, Tomography, X-Ray Computed, CT, fi brosis, interstitial lung abnormalities, interstitial lung disease, survey, ILA Study Group, fibrosis, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

BackgroundInterstitial lung abnormalities (ILA) may represent undiagnosed early-stage or subclinical interstitial lung disease (ILD). ILA are often observed incidentally in patients who subsequently develop clinically overt ILD. There is limited information on consensus definitions for, and the appropriate evaluation of, ILA. Early recognition of patients with ILD remains challenging, yet critically important. Expert consensus could inform early recognition and referral.Research questionCan consensus-based expert recommendations be identified to guide clinicians in the recognition, referral, and follow-up of patients with or at risk of developing early ILDs?Study design and methodsPulmonologists and radiologists with expertise in ILD participated in two iterative rounds of surveys. The surveys aimed to establish consensus regarding ILA reporting, identification of patients with ILA, and identification of populations that might benefit from screening for ILD. Recommended referral criteria and follow-up processes were also addressed. Threshold for consensus was defined a priori as ≥ 75% agreement or disagreement.ResultsFifty-five experts were invited and 44 participated; consensus was reached on 39 of 85 questions. The following clinically important statements achieved consensus: honeycombing and traction bronchiectasis or bronchiolectasis indicate potentially progressive ILD; honeycombing detected during lung cancer screening should be reported as potentially significant (eg, with the Lung CT Screening Reporting and Data System "S-modifier" [Lung-RADS; which indicates clinically significant or potentially significant noncancer findings]), recommending referral to a pulmonologist in the radiology report; high-resolution CT imaging and full pulmonary function tests should be ordered if nondependent subpleural reticulation, traction bronchiectasis, honeycombing, centrilobular ground-glass nodules, or patchy ground-glass opacity are observed on CT imaging; patients with honeycombing or traction bronchiectasis should be referred to a pulmonologist irrespective of diffusion capacity values; and patients with systemic sclerosis should be screened with pulmonary function tests for early-stage ILD.InterpretationGuidance was established for identifying clinically relevant ILA, subsequent referral, and follow-up. These results lay the foundation for developing practical guidance on managing patients with ILA.

Published
2022

9. The Role of Chest Imaging in Patient Management During the COVID-19 Pandemic A Multinational Consensus Statement From the Fleischner Society

Author

Rubin, Geoffrey D, Ryerson, Christopher J, Haramati, Linda B, Sverzellati, Nicola, Kanne, Jeffrey P, Raoof, Suhail, Schluger, Neil W, Volpi, Annalisa, Yim, Jae-Joon, Martin, Ian BK, Anderson, Deverick J, Kong, Christina, Altes, Talissa, Bush, Andrew, Desai, Sujal R, Goldin, Jonathan, Goo, Jin Mo, Humbert, Marc, Inoue, Yoshikazu, Kauczor, Hans-Ulrich, Luo, Fengming, Mazzone, Peter J, Prokop, Mathias, Remy-Jardin, Martine, Richeldi, Luca, Schaefer-Prokop, Cornelia M, Tomiyama, Noriyuki, Wells, Athol U, and Leung, Ann N

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Patient Safety, Lung, Prevention, Infection, Good Health and Well Being, Betacoronavirus, COVID-19, Coronavirus Infections, Diagnosis, Differential, Disease Progression, Early Diagnosis, Humans, International Cooperation, Pandemics, Patient Care Management, Pneumonia, Viral, Radiography, Thoracic, Respiratory Tract Diseases, SARS-CoV-2, Tomography, X-Ray Computed, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

With more than 900,000 confirmed cases worldwide and nearly 50,000 deaths during the first 3 months of 2020, the coronavirus disease 2019 (COVID-19) pandemic has emerged as an unprecedented health care crisis. The spread of COVID-19 has been heterogeneous, resulting in some regions having sporadic transmission and relatively few hospitalized patients with COVID-19 and others having community transmission that has led to overwhelming numbers of severe cases. For these regions, health care delivery has been disrupted and compromised by critical resource constraints in diagnostic testing, hospital beds, ventilators, and health care workers who have fallen ill to the virus exacerbated by shortages of personal protective equipment. Although mild cases mimic common upper respiratory viral infections, respiratory dysfunction becomes the principal source of morbidity and mortality as the disease advances. Thoracic imaging with chest radiography and CT are key tools for pulmonary disease diagnosis and management, but their role in the management of COVID-19 has not been considered within the multivariable context of the severity of respiratory disease, pretest probability, risk factors for disease progression, and critical resource constraints. To address this deficit, a multidisciplinary panel comprised principally of radiologists and pulmonologists from 10 countries with experience managing patients with COVID-19 across a spectrum of health care environments evaluated the utility of imaging within three scenarios representing varying risk factors, community conditions, and resource constraints. Fourteen key questions, corresponding to 11 decision points within the three scenarios and three additional clinical situations, were rated by the panel based on the anticipated value of the information that thoracic imaging would be expected to provide. The results were aggregated, resulting in five main and three additional recommendations intended to guide medical practitioners in the use of chest radiography and CT in the management of COVID-19.

Published
2020

10. A Systematically Derived Exposure Assessment Instrument for Chronic Hypersensitivity Pneumonitis

Author

Barnes, Hayley, Morisset, Julie, Molyneaux, Philip, Westall, Glen, Glaspole, Ian, Collard, Harold R, Collaborators, CHP Exposure Assessment, Antoniou, Katerina M, Barber, Christopher M, Behr, Jürgen, Bonella, Francesco, Corte, Tamera, Costabel, Ulrich, Cottin, Vincent, Crestani, Bruno, Dalphin, Jean-Charles, Flaherty, Kevin R, Goh, Nicole, Johannson, Kerri A, Kondoh, Yasuhiro, Lederer, David, Lee, Joyce, Maher, Toby M, Martinez, Fernando J, Morell, Ferran, Noth, Imre, Raghu, Ganesh, Renzoni, Elisabetta, Richeldi, Luca, Ryerson, Christopher J, Ryu, Jay H, Salisbury, Margaret L, Singh, Sheetu, Selman, Moises, Strek, Mary E, Tarlo, Susan M, Tomassetti, Sara, Vancheri, Carlo, Vasakova, Martina, Wolters, Paul, and Wells, Athol

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Clinical Research, Management of diseases and conditions, 7.3 Management and decision making, Alveolitis, Extrinsic Allergic, Chronic Disease, Consensus, Humans, Lung, Tomography, X-Ray Computed, extrinsic allergic alveolitis, hypersensitivity pneumonitis, interstitial lung diseases, CHP Exposure Assessment Collaborators, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

BackgroundChronic hypersensitivity pneumonitis (CHP) is an immune-mediated interstitial lung disease (ILD) caused by inhalational exposure to environmental antigens, resulting in parenchymal fibrosis. By definition, a diagnosis of CHP assumes a history of antigen exposure, but only half of all patients eventually diagnosed with CHP will have a causative antigen identified. Individual clinician variation in eliciting a history of antigen exposure may affect the frequency and confidence of CHP diagnosis.MethodsA list of potential causative exposures were derived from a systematic review of the literature. A Delphi method was applied to an international panel of ILD experts to obtain consensus regarding technique for the elicitation of exposure to antigens relevant to a diagnosis of CHP. The consensus threshold was set at 80% agreement, and median ≤ 2, interquartile range = 0 on a 5-point Likert scale (1, strongly agree; 2, tend to agree; 3, neither agree nor disagree; 4, disagree; 5, strongly disagree).ResultsIn two rounds, 36/40 experts participated. Experts agreed on 18 exposure items to ask every patient with suspected CHP. Themes included CHP inducing exposures, features that contribute to an exposure's relevance, and quantification of a relevant exposure. Based on the results from the literature review and Delphi process, a CHP exposure assessment instrument was derived. Using cognitive interviews, the instrument was revised by patients with ILD for readability and usability.ConclusionsThis Delphi survey provides items that ILD experts agree are important to ask in all patients presenting with suspected CHP and provides basis for a systematically derived CHP exposure assessment instrument. Clinical utility of this exposure assessment instrument may be affected by different local prevalence patterns of exposures. Ongoing research is required to clinically validate these items and consider their impact in more geographically diverse settings.

Published
2020

13. Fatigue in Covid-19 survivors: The potential impact of a nutritional supplement on muscle strength and function

Author

Steering Committee, Landi, Francesco, Gremese, Elisa, Coordination, Bernabei, Roberto, Fantoni, Massimo, Gasbarrini, Antonio, Field investigators, Gastroenterology team, Porcari, Serena, Settanni, Carlo Romano, Geriatric team, Benvenuto, Francesca, Bramato, Giulia, Brandi, Vincenzo, Carfì, Angelo, Ciciarello, Francesca, Fabrizi, Sofia, Galluzzo, Vincenzo, Lo Monaco, Maria Rita, Martone, Anna Maria, Marzetti, Emanuele, Napolitano, Carmen, Pagano, Francesco Cosimo, Pais, Cristina, Rocchi, Sara, Rota, Elisabetta, Salerno, Andrea, Tosato, Matteo, Tritto, Marcello, Zazzara, Maria Beatrice, Calvani, Riccardo, Catalano, Lucio, Picca, Anna, Savera, Giulia, Damiano, Francesco Paolo, Rocconi, Alessandra, Galliani, Alessandro, Spaziani, Giovanni, Tupputi, Salvatore, Cocchi, Camilla, Pirone, Flavia, D'Ignazio, Federica, Cacciatore, Stefano, Infectious disease team, Cauda, Roberto, Tamburrini, Enrica, Borghetti, A., Di Gianbenedetto, Simona, Murri, Rita, Cingolani, Antonella, Ventura, Giulio, Taddei, E., Moschese, D., Ciccullo, A., Dusina, A., Internal Medicine team, Stella, Leonardo, Addolorato, Giovanni, Franceschi, Francesco, Mingrone, Gertrude, Zocco, M.A., Microbiology team, Sanguinetti, Maurizio, Cattani, Paola, Marchetti, Simona, Posteraro, Brunella, Sali, M., Neurology team, Bizzarro, Alessandra, Lauria, Alessandra, Ophthalmology team, Rizzo, Stanislao, Savastano, Maria Cristina, Gambini, G., Cozzupoli, G.M., Culiersi, C., Otolaryngology team, Passali, Giulio Cesare, Paludetti, Gaetano, Galli, Jacopo, Crudo, F., Di Cintio, G., Longobardi, Y., Tricarico, L., Santantonio, M., Pediatric team, Buonsenso, Danilo, Valentini, P., Pata, D., Sinatti, D., De Rose, C., Pneumology team, Richeldi, Luca, Lombardi, Francesco, Calabrese, A., Leone, Paolo Maria, Calvello, Maria Rosaria, Intini, Enrica, Montemurro, Giuliano, Psychiatric team, Sani, Gabriele, Janiri, Delfina, Simonetti, Alessio, Giuseppin, G., Molinaro, M., odica, M., Radiology team, Natale, Luigi, Larici, Anna Rita, Marano, Riccardo, Rheumatology team, Paglionico, Annamaria, Petricca, Luca, Gigante, Luca, Natalello, G., Fedele, A.L., Lizzio, M.M., Tolusso, B., Di Mario, Clara, Alivernini, S., Vascular team, Santoliquido, Angelo, Santoro, Luca, Di Giorgio, Angela, Nesci, Antonio, and Popolla, V.

Published
2022
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14. A real-time integrated framework to support clinical decision making for covid-19 patients

Author

Murri, Rita, Masciocchi, Carlotta, Lenkowicz, Jacopo, Fantoni, Massimo, Damiani, Andrea, Marchetti, Antonio, Sergi, Paolo Domenico Angelo, Arcuri, Giovanni, Cesario, Alfredo, Patarnello, Stefano, Antonelli, Massimo, Bellantone, Rocco, Bernabei, Roberto, Boccia, Stefania, Calabresi, Paolo, Cambieri, Andrea, Cauda, Roberto, Colosimo, Cesare, Crea, Filippo, De Maria, Ruggero, De Stefano, Valerio, Franceschi, Francesco, Gasbarrini, Antonio, Landolfi, Raffaele, Parolini, Ornella, Richeldi, Luca, Sanguinetti, Maurizio, Urbani, Andrea, Zega, Maurizio, Scambia, Giovanni, and Valentini, Vincenzo

Published
2022
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15. Long-term evaluation of the safety and efficacy of recombinant human pentraxin-2 (rhPTX-2) in patients with idiopathic pulmonary fibrosis (IPF): an open-label extension study

Author

Raghu, Ganesh, Hamblin, Mark J., Brown, A. Whitney, Golden, Jeffrey A., Ho, Lawrence A., Wijsenbeek, Marlies S., Vasakova, Martina, Pesci, Alberto, Antin-Ozerkis, Danielle E., Meyer, Keith C., Kreuter, Michael, Burgess, Tracy, Kamath, Nikhil, Donaldson, Francis, and Richeldi, Luca

Published
2022
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16. Ventilatory Support in Patients with COVID-19

Author

Leone, Paolo Maria, Siciliano, Matteo, Simonetti, Jacopo, Lopez, Angelena, Zaman, Tanzira, Varone, Francesco, Richeldi, Luca, Crusio, Wim E., Series Editor, Dong, Haidong, Series Editor, Radeke, Heinfried H., Series Editor, Rezaei, Nima, Series Editor, and Xiao, Junjie, Series Editor

Published
2021
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17. Detection and Early Referral of Patients With Interstitial Lung Abnormalities: An Expert Survey Initiative

Author

Abtin, Fereidoun, Antoniou, Katerina, Blackwell, Timothy, Brown, Kevin, Chung, Jonathan, Corte, Tamera, Crestani, Bruno, Crossno, Peter, Culver, Daniel, de Andrade, Joao, Deveraj, Anand, Flaherty, Kevin, Gudmundsson, Gunnar, Hatabu, Hiroto, Jacob, Joe, Johansson, Kerri, Kanne, Jeff, Kazerooni, Ella, Kolb, Martin, Lynch, David, Maher, Toby, Martinez, Fernando, Morais, Antonio, Nathan, Steven D., Noth, Imre, Oldham, Justin, Podolanczuk, Anna, Poletti, Venerino, Ravaglia, Claudia, Renzoni, Elizabetta, Richeldi, Luca, Rubin, Geoffrey, Ryerson, Chris, Sahoo, Debasis, Tomassetti, Sara, Spagnolo, Paolo, Strek, Mary E., Suh, Rob, Sverzellati, Nicola, Valeyre, Dominique, Walsh, Simon, Washko, George, White, Eric S., Hunninghake, Gary M., Goldin, Jonathan G., Kadoch, Michael A., Kropski, Jonathan A., Rosas, Ivan O., Wells, Athol U., Yadav, Ruchi, Lazarus, Howard M., Abtin, Fereidoun G., Corte, Tamera J., de Andrade, Joao A., Johannson, Kerri A., Kolb, Martin R., Lynch, David A., Oldham, Justin M., and Washko, George R.

Published
2022
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18. The Role of Inflammation and Fibrosis in Interstitial Lung Disease Treatment Decisions.

Author

Behr, Juergen, Salisbury, Margaret L., Walsh, Simon L. F., Podolanczuk, Anna J., Hariri, Lida P., Hunninghake, Gary M., Kolb, Martin, Ryerson, Christopher J., Cottin, Vincent, Beasley, Mary B., Corte, Tamera, Glanville, Allan R., Adegunsoye, Ayodeji, Hogaboam, Cory, Wuyts, Wim A., Noth, Imre, Oldham, Justin M., Richeldi, Luca, Raghu, Ganesh, and Wells, Athol U.

Subjects
PULMONARY fibrosis, INTERSTITIAL lung diseases, THERAPEUTICS, IDIOPATHIC diseases, IDIOPATHIC pulmonary fibrosis, MONONUCLEAR leukocytes
Abstract

The article summarizes knowledge on inflammation and fibrosis in interstitial lung disease (ILD), with the relative burden of each in an individual patient gleaned by considering several domains, including clinical history and lung function, imaging, laboratory including bronchoalveolar lavage (BAL), and histopathology. Topics discussed include prediction of disease behavior and first-line treatment decision, inflammation in ILD, and fibrosis in ILD.

Published
2024
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19. Pamrevlumab for Idiopathic Pulmonary Fibrosis: The ZEPHYRUS-1 Randomized Clinical Trial.

Author

Raghu, Ganesh, Richeldi, Luca, Fernández Pérez, Evans R., De Salvo, Maria Cristina, Silva, Rafael S., Song, Jin Woo, Ogura, Takashi, Xu, Zuo Jun, Belloli, Elizabeth A., Zhang, Xueping, Seid, Lorilyn L., and Poole, Lona

Subjects
*IDIOPATHIC pulmonary fibrosis, *CONNECTIVE tissue growth factor, *PULMONARY fibrosis, *VITAL capacity (Respiration), *PATIENT compliance, *CLINICAL trials, *ACOUSTIC radiation force impulse imaging, *LIVER histology
Abstract

Key Points: Question: Does the connective tissue growth factor inhibitor pamrevlumab decrease the rate of decline in lung function for patients with idiopathic pulmonary fibrosis compared with placebo? Findings: In this phase 3 randomized clinical trial including 356 adults with idiopathic pulmonary fibrosis, the decline in forced vital capacity at 48 weeks did not differ significantly between patients treated with pamrevlumab (–260 mL) vs placebo (–330 mL). Meaning: Use of pamrevlumab among patients with idiopathic pulmonary fibrosis did not result in a difference in decline in forced vital capacity from baseline to 48 weeks compared with placebo. Importance: Current treatments for idiopathic pulmonary fibrosis slow the rate of lung function decline, but may be associated with adverse events that affect medication adherence. In phase 2 trials, pamrevlumab (a fully human monoclonal antibody that binds to and inhibits connective tissue growth factor activity) attenuated the progression of idiopathic pulmonary fibrosis without substantial adverse events. Objective: To assess the efficacy and safety of pamrevlumab for patients with idiopathic pulmonary fibrosis. Design, Setting, and Participants: Phase 3 randomized clinical trial including 356 patients aged 40 to 85 years with idiopathic pulmonary fibrosis who were not receiving antifibrotic treatment with nintedanib or pirfenidone at enrollment. Patients were recruited from 117 sites in 9 countries between July 18, 2019, and July 29, 2022; the last follow-up encounter occurred on August 28, 2023. Interventions: Pamrevlumab (30 mg/kg administered intravenously every 3 weeks; n = 181) or placebo (n = 175) for 48 weeks. Main Outcomes and Measures: The primary outcome was absolute change in forced vital capacity (FVC) from baseline to week 48. There were 5 secondary outcomes (including time to disease progression, which was defined as a decline of ≥10% in predicted FVC or death). The exploratory outcomes included patient-reported symptoms. Adverse events were reported. Results: Among 356 patients (mean age, 70.5 years; 258 [72.5%] were men; 221 [62.1%] were White), 277 (77.8%) completed the trial. There was no significant between-group difference for absolute change in FVC from baseline to week 48 (least-squares mean, −260 mL [95% CI, −350 to −170 mL] in the pamrevlumab group vs −330 mL [95% CI, −430 to −230 mL] in the placebo group; mean between-group difference, 70 mL [95% CI, −60 to 190 mL], P =.29). There were no significant between-group differences in any of the secondary outcomes or in the patient-reported outcomes. In the pamrevlumab group, there were 160 patients (88.4%) with treatment-related adverse events and 51 patients (28.2%) with serious adverse events vs 151 (86.3%) and 60 (34.3%), respectively, in the placebo group. During the study, 23 patients died in each group (12.7% in the pamrevlumab group vs 13.1% in the placebo group). Conclusions and Relevance: Among patients with idiopathic pulmonary fibrosis treated with pamrevlumab or placebo, there was no statistically significant between-group difference for the primary outcome of absolute change in FVC from baseline to week 48. Trial Registration: ClinicalTrials.gov Identifier: NCT03955146 This randomized clinical trial compares the efficacy and safety of pamrevlumab vs placebo for patients with idiopathic pulmonary fibrosis. [ABSTRACT FROM AUTHOR]

Published
2024
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20. Identification of Diagnostic Criteria for Chronic Hypersensitivity Pneumonitis. An International Modified Delphi Survey

Author

Morisset, Julie, Johannson, Kerri A, Jones, Kirk D, Wolters, Paul J, Collard, Harold R, Walsh, Simon LF, Ley, Brett, Antoniou, Katerina M, Assayag, Deborah, Behr, Juergen, Bonella, Francesco, Brown, Kevin K, Collins, Bridget F, Cormier, Yvon, Corte, Tamera J, Costabel, Ulrich, Danoff, Sonye K, de Boer, Kaïssa, Fernandez Perez, Evans R, Flaherty, Kevin R, Goh, Nicole SL, Glaspole, Ian, Jones, Mark G, Kondoh, Yasuhiro, Kreuter, Michael, Lacasse, Yves, Lancaster, Lisa H, Lederer, David J, Lee, Joyce S, Maher, Toby M, Martinez, Fernando J, Meyer, Keith C, Mooney, Joshua J, Gall, Xavier Muñoz, Noble, Paul W, Noth, Imre, Oldham, Justin M, Alberto de Castro Pereira, Carlos, Poletti, Venerino, Selman, Moises, Spagnolo, Paolo, Renzoni, Elisabetta, Richeldi, Luca, Ryerson, Christopher J, Ryu, Jay H, Salisbury, Margaret L, Strek, Mary E, Tomassetti, Sara, Valeyre, Dominique, Vancheri, Carlo, Wijsenbeek, Marlies S, and Wuyts, Wim

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Detection, screening and diagnosis, 4.1 Discovery and preclinical testing of markers and technologies, Alveolitis, Extrinsic Allergic, Chronic Disease, Consensus, Delphi Technique, Female, Humans, Internationality, Male, Surveys and Questionnaires, hypersensitivity pneumonitis, interstitial lung disease, diagnosis, Delphi, HP Delphi Collaborators, Diagnosis, Hypersensitivity pneumonitis, Interstitial Lung Disease, Medical and Health Sciences, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

RationaleCurrent diagnosis of chronic hypersensitivity pneumonitis (cHP) involves considering a combination of clinical, radiological, and pathological information in multidisciplinary team discussions. However, this approach is highly variable with poor agreement between centers.ObjectivesWe aimed to identify diagnostic criteria for cHP that reach consensus among international experts.MethodsA 3-round modified Delphi survey was conducted between April and August 2017. Forty-five experts in interstitial lung disease from 14 countries participated in the online survey. Diagnostic items included in round 1 were generated using expert interviews and literature review. During rounds 1 and 2, experts rated the importance of each diagnostic item on a 5-point Likert scale. The a priori threshold of consensus was ≥ 75% of experts rating a diagnostic item as very important or important. In the third round, experts graded the items that met consensus as important and provided their level of diagnostic confidence for a series of clinical scenarios.Measurements and main resultsConsensus was achieved on 18 of the 40 diagnostic items. Among these, experts gave the highest level of importance to the identification of a causative antigen, time relation between exposure and disease, mosaic attenuation on chest imaging, and poorly formed non-necrotizing granulomas on pathology. In clinical scenarios, the diagnostic confidence of experts in cHP was heightened by the presence of these diagnostic items.ConclusionThis consensus-based approach for the diagnosis of cHP represents a first step towards the development of international guidelines for the diagnosis of cHP.

Published
2018

21. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

Author

Wolters, Paul J, Blackwell, Timothy S, Eickelberg, Oliver, Loyd, James E, Kaminski, Naftali, Jenkins, Gisli, Maher, Toby M, Molina-Molina, Maria, Noble, Paul W, Raghu, Ganesh, Richeldi, Luca, Schwarz, Marvin I, Selman, Moises, Wuyts, Wim A, and Schwartz, David A

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Rare Diseases, Autoimmune Disease, Lung, Aetiology, 2.1 Biological and endogenous factors, Respiratory, Fibroblasts, Humans, Idiopathic Pulmonary Fibrosis, Risk Factors, Clinical Sciences, Public Health and Health Services, Other Medical and Health Sciences, Cardiovascular medicine and haematology, Clinical sciences
Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. Although understanding of the pathogenic mechanisms continues to evolve, evidence indicates that distal airway and alveolar epithelial cells are central drivers of the disease. In this Viewpoint, we review the history of naming and classifications used to define the disease now referred to as IPF, in the context of understanding the clinical presentation, causes, and pathogenesis of the disease. We aim to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. To initiate this discussion, we offer new suggestions to update the name of this disease and new approaches to classify all forms of pulmonary fibrosis.

Published
2018

23. Meaningful Endpoints for Idiopathic Pulmonary Fibrosis (IPF) Clinical Trials: Emphasis on ‘Feels, Functions, Survives’. Report of a Collaborative Discussion in a Symposium with Direct Engagement from Representatives of Patients, Investigators, the National Institutes of Health, a Patient Advocacy Organization, and a Regulatory Agency

Author

Raghu, Ganesh, primary, Ghazipura, Marya, additional, Fleming, Thomas R., additional, Aronson, Kerri I., additional, Behr, Jürgen, additional, Brown, Kevin K., additional, Flaherty, Kevin R., additional, Kazerooni, Ella A., additional, Maher, Toby M., additional, Richeldi, Luca, additional, Lasky, Joseph A., additional, Swigris, Jeffrey J., additional, Busch, Robert, additional, Garrard, Lili, additional, Ahn, Dong-Hyun, additional, Li, Ji, additional, Puthawala, Khalid, additional, Rodal, Gabriela, additional, Seymour, Sally, additional, Weir, Nargues, additional, Danoff, Sonye K., additional, Ettinger, Neil, additional, Goldin, Jonathan, additional, Glassberg, Marilyn K., additional, Kawano-Dourado, Leticia, additional, Khalil, Nasreen, additional, Lancaster, Lisa, additional, Lynch, David A., additional, Mageto, Yolanda, additional, Noth, Imre, additional, Shore, Jessica E., additional, Wijsenbeek, Marlies, additional, Brown, Robert, additional, Grogan, Daniel, additional, Ivey, Dorothy, additional, Golinska, Patrycja, additional, Karimi-Shah, Banu, additional, and Martinez, Fernando J., additional

Published
2024
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24. The Role of Inflammation and Fibrosis in ILD Treatment Decisions

Author

Behr, Juergen, primary, Salisbury, Margaret L, additional, Walsh, Simon L. F., additional, Podolanczuk, Anna J., additional, Hariri, Lida P, additional, Hunninghake, Gary M, additional, Kolb, Martin, additional, Ryerson, Christopher J, additional, Cottin, Vincent, additional, Beasley, Mary B., additional, Corte, Tamera, additional, Glanville, Allan R, additional, Adegunsoye, Ayodeji, additional, Hogaboam, Cory, additional, Wuyts, Wim A., additional, Noth, Imre, additional, Oldham, Justin M., additional, Richeldi, Luca, additional, Raghu, Ganesh, additional, and Wells, Athol U., additional

Published
2024
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25. Vaccines and Myocardial Injury in Patients Hospitalized for COVID-19 Infection: the CardioCOVID-Gemelli Study

Author

Montone, Rocco Antonio, primary, Rinaldi, Riccardo, additional, Masciocchi, Carlotta, additional, Lilli, Livia, additional, Damiani, Andrea, additional, La Vecchia, Giulia, additional, Iannaccone, Giulia, additional, Basile, Mattia, additional, Salzillo, Carmine, additional, Caff, Andrea, additional, Bonanni, Alice, additional, De Pascale, Gennaro, additional, Grieco, Domenico Luca, additional, Tanzarella, Eloisa Sofia, additional, Buonsenso, Danilo, additional, Murri, Rita, additional, Fantoni, Massimo, additional, Liuzzo, Giovanna, additional, Sanna, Tommaso, additional, Richeldi, Luca, additional, Sanguinetti, Maurizio, additional, Massetti, Massimo, additional, Trani, Carlo, additional, Tshomba, Yamume, additional, Gasbarrini, Antonio, additional, Valentini, Vincenzo, additional, Antonelli, Massimo, additional, and Crea, Filippo, additional

Published
2024
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26. Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial

Author

Richeldi, Luca, primary, Schiffman, Courtney, additional, Behr, Jürgen, additional, Inoue, Yoshikazu, additional, Corte, Tamera J, additional, Cottin, Vincent, additional, Jenkins, R. Gisli, additional, Nathan, Steven D, additional, Raghu, Ganesh, additional, Walsh, Simon L. F., additional, Jayia, Parminderjit K., additional, Kamath, Nikhil, additional, and Martinez, Fernando J., additional

Published
2024
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27. TRITRIAL: The Impact of Fixed Triple Therapy with Beclometasone/Formoterol/Glycopyrronium on Health Status and Adherence in Chronic Obstructive Pulmonary Disease in an Italian Context of Real Life

Author

Richeldi, Luca, primary, Schino, Pietro, additional, Bargagli, Elena, additional, Ricci, Alberto, additional, Rocca, Alberto, additional, Marchesani, Francesca, additional, Pennisi, Alfio, additional, Camiciottoli, Gianna, additional, D'Amato, Maria, additional, Macagno, Francesco, additional, Scaffidi Argentina, Ulrica, additional, Ingrassia, Eleonora, additional, and Piraino, Alessio, additional

Published
2024
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28. Severe asthma: One disease and multiple definitions

Author

Guarnieri, Gabriella, Patella, Vincenzo, Maria Pia, Foschino Barbaro, Carpagnano, Giovanna Elisiana, Colle, Anna del, Scioscia, Giulia, Gerolamo, Pelaia, Puggioni, Francesca, Racca, Francesca, Favero, Elisabetta, Iannacone, Sandra, Savi, Eleonora, Montagni, Marcello, Camiciottoli, Gianna, Allegrini, Chiara, Lombardi, Carlo, Spadaro, Giuseppe, Detoraki, Caterina, Menzella, Francesco, Galeone, Carla, Ruggiero, Patrizia, Yacoub, Monna Rita, Berti, Alvise, Scichilone, Nicola, Durante, Carmen, Costantino, Maria Teresa, Roncallo, Chiara, Braschi, Mariachiara, D’Adda, Alice, Ridolo, Erminia, Triggiani, Massimo, Parente, Roberta, Maria, D’Amato, Verrillo, Maria Vittoria, Rolla, Giovanni, Brussino, Luisa, Frazzetto, Agata Valentina, Cristina, Zappa Maria, Lilli, Marianna, Crimi, Nunzio, Bonavia, Marco, Corsico, Angelo Guido, Grosso, Amelia, Del Giacco, Stefano, Deidda, Margherita, Ricciardi, Luisa, Isola, Stefania, Cicero, Francesca, Amato, Giuliana, Vita, Federica, Spanevello, Antonio, Pignatti, Patrizia, Cherubino, Francesca, Visca, Dina, Massimo Ricciardolo, Fabio Luigi, Anna Carriero, Vitina Maria, Bertolini, Francesca, Santus, Pierachille, Barlassina, Roberta, Airoldi, Andrea, Guida, Giuseppe, Eleonora, Nucera, Aruanno, Arianna, Rizzi, Angela, Caruso, Cristiano, Colantuono, Stefania, Senna, Gianenrico, Caminati, Marco, Arcolaci, Alessandra, Vianello, Andrea, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Centanni, Stefano, Luraschi, Simone, Ruggeri, Silvia, Rinaldo, Rocco, Parazzini, Elena, Calabrese, Cecilia, Flora, Martina, Cosmi, Lorenzo, Di Pietro, Linda, Maggi, Enrico, Pini, Laura, Macchia, Luigi, Di Bona, Danilo, Richeldi, Luca, Condoluci, Carola, Fuso, Leonello, Bonini, Matteo, Farsi, Alessandro, Carli, Giulia, Montuschi, Paolo, Santini, Giuseppe, Conte, Maria Elisabetta, Turchet, Elisa, Barbetta, Carlo, Mazza, Francesco, D’Alo, Simona, Pucci, Stefano, Caiaffa, Maria Filomena, Minenna, Elena, D'Elia, Luciana, Pasculli, Carlo, Viviano, Vittorio, Tarsia, Paolo, Rolo, Joyce, Di Proietto, Mariacarmela, Lo Cicero, Salvatore, Bagnasco, Diego, Paggiaro, Pierluigi, Latorre, Manuela, Folli, Chiara, Testino, Elisa, Bassi, Arianna, Milanese, Manlio, Heffler, Enrico, Manfredi, Andrea, Riccio, Anna Maria, De Ferrari, Laura, Blasi, Francesco, Canevari, Rikki Frank, Canonica, Giorgio Walter, and Passalacqua, Giovanni

Published
2021
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31. Economic impact of mepolizumab in uncontrolled severe eosinophilic asthma, in real life

Author

Guarnieri, Gabriella, Patella, Vincenzo, Maria Pia, Foschino Barbaro, Carpagnano, Elisiana, Colle, Anna del, Scioscia, Giulia, Gerolamo, Pelaia, Paggiaro, Pierluigi, Latorre, Manuela, Puggioni, Francesca, Racca, Francesca, Favero, Elisabetta, Iannacone, Sandra, Savi, Eleonora, Montagni, Marcello, Camiciottoli, Gianna, Allegrini, Chiara, Spadaro, Giuseppe, Detoraki, Caterina, Galeone, Carla, Ruggiero, Patrizia, Yacoub, Monna Rita, Berti, Alvise, Colombo, Gisella, Scichilone, Nicola, Durante, Carmen, Costantino, Maria Teresa, Roncallo, Chiara, Braschi, Mariachiara, Blasi, Francesco, D'Adda, Alice, Ridolo, Erminia, Triggiani, Massimo, Parente, Roberta, Maria, D'Amato, Verrillo, Maria Vittoria, Cristina, Zappa Maria, Lilli, Marianna, Crimi, Nunzio, Bonavia, Marco, Corsico, Angelo Guido, Grosso, Amelia, Del Giacco, Stefano, Deidda, Margherita, Ricciardi, Luisa, Isola, Stefania, Cicero, Francesca, Amato, Giuliana, Vita, Federica, Spanevello, Antonio, Pignatti, Patrizia, Cherubino, Francesca, Visca, Dina, Aletti, Eleonora, Massimo Ricciardolo, Fabio Luigi, Anna Carriero, Vitina Maria, Bertolini, Francesca, Santus, Pierachille, Barlassina, Roberta, Airoldi, Andrea, Guida, Giuseppe, Eleonora, Nucera, Aruanno, Arianna, Rizzi, Angela, Caruso, Cristiano, Colantuono, Stefania, Arcolaci, Alessandra, Vianello, Andrea, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Centanni, Stefano, Luraschi, Simone, Ruggeri, Silvia, Rinaldo, Rocco, Parazzini, Elena, Calabrese, Cecilia, Flora, Martina, Cosmi, Lorenzo, Di Pietro, Linda, Maggi, Enrico, Pini, Laura, Macchia, Luigi, Di Bona, Danilo, Richeldi, Luca, Condoluci, Carola, Fuso, Leonello, Bonini, Matteo, Farsi, Alessandro, Carli, Giulia, Montuschi, Paolo, Santini, Giuseppe, Conte, Maria Elisabetta, Turchet, Elisa, Barbetta, Carlo, Mazza, Francesco, D'Alo, Simona, Pucci, Stefano, Caiaffa, Maria Filomena, Minenna, Elena, D'Elia, Luciana, Pasculli, Carlo, Viviano, Vittorio, Tarsia, Paolo, Rolo, Joyce, Di Proietto, Mariacarmela, Lo Cicero, Salvatore, Bagnasco, Diego, Povero, Massimiliano, Pradelli, Lorenzo, Brussino, Luisa, Rolla, Giovanni, Caminati, Marco, Menzella, Francesco, Heffler, Enrico, Canonica, Giorgio Walter, Senna, Gianenrico, Milanese, Manlio, Lombardi, Carlo, Bucca, Caterina, Manfredi, Andrea, Canevari, Rikki Frank, and Passalacqua, Giovanni

Published
2021
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32. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease

Author

Flaherty, Kevin R, Brown, Kevin K, Wells, Athol U, Clerisme-Beaty, Emmanuelle, Collard, Harold R, Cottin, Vincent, Devaraj, Anand, Inoue, Yoshikazu, Le Maulf, Florence, Richeldi, Luca, Schmidt, Hendrik, Walsh, Simon, Mezzanotte, William, and Schlenker-Herceg, Rozsa

Subjects
Lung, Autoimmune Disease, Clinical Trials and Supportive Activities, Clinical Research, Rare Diseases, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, Respiratory, Good Health and Well Being, interstitial fibrosis, rare lung diseases
Abstract

600 patients aged ≥18 years will be randomised in a 1:1 ratio to nintedanib or placebo. Patients with diagnosis of IPF will be excluded. The study population will be enriched with two-thirds having a usual interstitial pneumonia-like pattern on HRCT. The primary endpoint is the annual rate of decline in forced vital capacity over 52 weeks. The main secondary endpoints are the absolute change from baseline in King's Brief Interstitial Lung Disease Questionnaire total score, time to first acute interstitial lung disease exacerbation or death and time to all-cause mortality over 52 weeks.Ethics and disseminationThe trial is conducted in accordance with the Declaration of Helsinki, the International Conference on Harmonisation Tripartite Guideline for Good Clinical Practice (GCP) and Japanese GCP regulations.Trial registration numberNCT02999178.

Published
2017

33. The diagnosis of idiopathic pulmonary fibrosis: current and future approaches

Author

Martinez, Fernando J, Chisholm, Alison, Collard, Harold R, Flaherty, Kevin R, Myers, Jeffrey, Raghu, Ganesh, Walsh, Simon LF, White, Eric S, and Richeldi, Luca

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Clinical Research, Biomedical Imaging, Lung, 4.2 Evaluation of markers and technologies, Detection, screening and diagnosis, 4.1 Discovery and preclinical testing of markers and technologies, Respiratory, Biopsy, Diagnosis, Differential, Humans, Idiopathic Pulmonary Fibrosis, Symptom Assessment, Tomography, X-Ray Computed, Public Health and Health Services, Other Medical and Health Sciences, Cardiovascular medicine and haematology, Clinical sciences
Abstract

With the recent development of two effective treatments for patients with idiopathic pulmonary fibrosis, an accurate diagnosis is crucial. The traditional approach to diagnosis emphasises the importance of thorough clinical and laboratory evaluations to exclude secondary causes of disease. High-resolution CT is a critical initial diagnostic test and acts as a tool to identify patients who should undergo surgical lung biopsy to secure a definitive histological diagnosis of usual interstitial pneumonia pattern. This diagnostic approach faces several challenges. Many patients with suspected idiopathic pulmonary fibrosis present with atypical high-resolution CT characteristics but are unfit for surgical lung biopsy, therefore preventing a confident diagnosis. The state of the art suggests an iterative, multidisciplinary process that incorporates available clinical, laboratory, imaging, and histological features. Recent research has explored genomic techniques to molecularly phenotype patients with interstitial lung disease. In the future, clinicians will probably use blood-specific or lung-specific molecular markers in combination with other clinical, physiological, and imaging features to enhance diagnostic efforts, refine prognostic recommendations, and influence the initial or subsequent treatment options. There is an urgent and increasing need for well designed, large, prospective studies measuring the effect of different diagnostic approaches. Ultimately, this will help to inform the development of guidelines and tailor clinical practice for the benefit of patients.

Published
2017

35. Sarilumab use in severe SARS-CoV-2 pneumonia

Author

Gremese, Elisa, Cingolani, Antonella, Bosello, Silvia Laura, Alivernini, Stefano, Tolusso, Barbara, Perniola, Simone, Landi, Francesco, Pompili, Maurizio, Murri, Rita, Santoliquido, Angelo, Garcovich, Matteo, Sali, Michela, De Pascale, Gennaro, Gabrielli, Maurizio, Biscetti, Federico, Montalto, Massimo, Tosoni, Alberto, Gambassi, Giovanni, Rapaccini, Gian Ludovico, Iaconelli, Amerigo, Zileri Del Verme, Lorenzo, Petricca, Luca, Fedele, Anna Laura, Lizzio, Marco Maria, Tamburrini, Enrica, Natalello, Gerlando, Gigante, Laura, Bruno, Dario, Verardi, Lucrezia, Taddei, Eleonora, Calabrese, Angelo, Lombardi, Francesco, Bernabei, Roberto, Cauda, Roberto, Franceschi, Francesco, Landolfi, Raffaele, Richeldi, Luca, Sanguinetti, Maurizio, Fantoni, Massimo, Antonelli, Massimo, and Gasbarrini, Antonio

Published
2020
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36. Oral CorticoSteroid sparing with biologics in severe asthma: A remark of the Severe Asthma Network in Italy (SANI)

Author

Canonica, Giorgio Walter, Blasi, Francesco, Paggiaro, Pierluigi, Senna, Gianenrico, Passalacqua, Giovanni, Spanevello, Antonio, Aliberti, Stefano, Bagnasco, Diego, Bonavia, Marco, Bonini, Matteo, Brussino, Luisa, Bucca, Caterina, Caiaffa, Maria F., Calabrese, Cecilia, Camiciottoli, Gianna, Caminati, Marco, Carpagnano, Giovanna E., Caruso, Cristiano, Centanni, Stefano, Conte, Maria E., Corsico, Angelo G., Cosmi, Lorenzo, Costantino, Maria T., Crimi, Nunzio, D’Alò, Simona, D'Amato, Maria, Del Giacco, Stefano, Farsi, Alessandro, Favero, Elisabetta, Foschino Barbaro, Maria P., Guarnieri, Gabriella, Guida, Giuseppe, Latorre, Manuela, Lo Cicero, Salvatore, Lombardi, Carlo, Macchia, Luigi, Mazza, Francesco, Menzella, Francesco, Milanese, Manlio, Montagni, Marcello, Montuschi, Paolo, Nucera, Eleonora, Parente, Roberta, Patella, Vincenzo, Pelaia, Girolamo, Pini, Laura, Puggioni, Francesca, Ricciardi, Luisa, Ricciardolo, Fabio L.M., Richeldi, Luca, Ridolo, Erminia, Rolla, Giovanni, Santus, Pierachille, Scichilone, Nicola, Spadaro, Giuseppe, Vianello, Andrea, Viviano, Vittorio, Yacoub, Mona R., Zappa, Maria C., and Heffler, Enrico

Published
2020
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39. Pharmacologic Treatment of IPF

Author

Smargiassi, Andrea, Pasciuto, Giuliana, Conte, Emanuele Giovanni, Andreani, Mariarita, Marra, Roberta, Richeldi, Luca, Rounds, Sharon I.S., Series Editor, Dixon, Anne, Series Editor, Schnapp, Lynn M., Series Editor, Meyer, Keith C., editor, and Nathan, Steven D., editor

Published
2019
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42. Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society

Author

Hatabu, Hiroto, Hunninghake, Gary M, Richeldi, Luca, Brown, Kevin K, Wells, Athol U, Remy-Jardin, Martine, Verschakelen, Johny, Nicholson, Andrew G, Beasley, Mary B, Christiani, David C, San José Estépar, Raúl, Seo, Joon Beom, Johkoh, Takeshi, Sverzellati, Nicola, Ryerson, Christopher J, Graham Barr, R, Goo, Jin Mo, Austin, John H M, Powell, Charles A, Lee, Kyung Soo, Inoue, Yoshikazu, and Lynch, David A

Published
2020
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43. A Systematically Derived Exposure Assessment Instrument for Chronic Hypersensitivity Pneumonitis

Author

Antoniou, Katerina M., Barber, Christopher M., Behr, Jürgen, Bonella, Francesco, Corte, Tamera, Costabel, Ulrich, Cottin, Vincent, Crestani, Bruno, Dalphin, Jean-Charles, Flaherty, Kevin R., Goh, Nicole, Johannson, Kerri A., Kondoh, Yasuhiro, Lederer, David, Lee, Joyce, Maher, Toby M., Martinez, Fernando J., Morell, Ferran, Noth, Imre, Raghu, Ganesh, Renzoni, Elisabetta, Richeldi, Luca, Ryerson, Christopher J., Ryu, Jay H., Salisbury, Margaret L., Singh, Sheetu, Selman, Moises, Strek, Mary E., Tarlo, Susan M., Tomassetti, Sara, Vancheri, Carlo, Vasakova, Martina, Wolters, Paul, Wells, Athol, Barnes, Hayley, Morisset, Julie, Molyneaux, Philip, Westall, Glen, Glaspole, Ian, and Collard, Harold R.

Published
2020
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44. 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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45. Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial

Author

Richeldi, Luca, Fernández Pérez, Evans R, Costabel, Ulrich, Albera, Carlo, Lederer, David J, Flaherty, Kevin R, Ettinger, Neil, Perez, Rafael, Scholand, Mary Beth, Goldin, Jonathan, Peony Yu, Kin-Hung, Neff, Thomas, Porter, Seth, Zhong, Ming, Gorina, Eduard, Kouchakji, Elias, and Raghu, Ganesh

Published
2020
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47. Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis

Author

Brownell, Robert, Kaminski, Naftali, Woodruff, Prescott G, Bradford, Williamson Z, Richeldi, Luca, Martinez, Fernando J, and Collard, Harold R

Subjects
Rare Diseases, Genetics, Clinical Research, Lung, Mind and Body, Clinical Trials and Supportive Activities, Good Health and Well Being, Humans, Idiopathic Pulmonary Fibrosis, Precision Medicine, idiopathic pulmonary fibrosis, stratified medicine, precision medicine, endotypes, biological markers, Medical and Health Sciences, Respiratory System
Abstract

Precision medicine is defined by the National Institute of Health's Precision Medicine Initiative Working Group as an approach to disease treatment that takes into account individual variability in genes, environment, and lifestyle. There has been increased interest in applying the concept of precision medicine to idiopathic pulmonary fibrosis, in particular to search for genetic and molecular biomarker-based profiles (so called endotypes) that identify mechanistically distinct disease subgroups. The relevance of precision medicine to idiopathic pulmonary fibrosis is yet to be established, but we believe that it holds great promise to provide targeted and highly effective therapies to patients. In this manuscript, we describe the field's nascent efforts in genetic/molecular endotype identification and how environmental and behavioral subgroups may also be relevant to disease management.

Published
2016

48. Approach to Clinical Trials for the Prevention of Pulmonary Fibrosis

Author

Kim, John S., primary, Montesi, Sydney B., additional, Adegunsoye, Ayodeji, additional, Humphries, Stephen M., additional, Salisbury, Margaret L., additional, Hariri, Lida P., additional, Kropski, Jonathan A., additional, Richeldi, Luca, additional, Wells, Athol U., additional, Walsh, Simon, additional, Jenkins, R. Gisli, additional, Rosas, Ivan, additional, Noth, Imre, additional, Hunninghake, Gary M., additional, Martinez, Fernando J., additional, and Podolanczuk, Anna J., additional

Published
2023
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