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1,886 results on '"Wells, Athol U."'

51. Defining genetic risk factors for scleroderma-associated interstitial lung disease: IRF5 and STAT4 gene variants are associated with scleroderma while STAT4 is protective against scleroderma-associated interstitial lung disease

Author

Stock, Carmel J. W., De Lauretis, Angelo, Visca, Dina, Daccord, Cecile, Kokosi, Maria, Kouranos, Vasilis, Margaritopoulos, George, George, Peter M., Molyneaux, Philip L., Nihtyanova, Svetlana, Chua, Felix, Maher, Toby M., Ong, Voon, Abraham, David J., Denton, Christopher P., Wells, Athol U., Wain, Louise V., and Renzoni, Elisabetta A.

Published
2020
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52. Rare pulmonary diseases and orphan drugs: where do we stand and where are we going to?

Author

Harari, Sergio, Humbert, Marc, Blasi, Francesco, Collard, Harold R, Matucci-Cerinic, Marco, Simonneau, Gerald, Vancheri, Carlo, Wells, Athol U, and Cottin, Vincent

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Rare Diseases, Lung, Pediatric, Chronic Obstructive Pulmonary Disease, Orphan Drug, Respiratory, Biomedical Research, Forecasting, Humans, Lung Diseases, Orphan Drug Production, Respiratory System Agents, Medical Physiology, Respiratory System, Cardiovascular medicine and haematology
Abstract

Updates on rare pulmonary diseases from the 6th International Meeting on Pulmonary Rare Diseases and Orphan Drugs http://ow.ly/PYUCC

Published
2015

53. Breaking barriers: holistic assessment of ability to work in patients with sarcoidosis

Author

Goh, Nicole, Holland, Anne, Hochreiter, Johann, Kock, Selene, Verheyen, Karolien, Verbraecken, Johan, Cantin, André, Gillson, Ashley, Hambly, Nathan, Kolb, Martin, Bendstrup, Elisabeth, Leistiko, Anna, Møller, Janne, Kaarteenaho, Riitta, Crestani, Bruno, Israël-Biet, Dominique, Cohen Aubart, Fleur, Nunes, Hilario, Valeyre, Dominique, Bonella, Francesco, Buschulte, Katharina, Costabel, Ulrich, Kortsen, Peter, Kreuter, Michael, Luedike, Peter, Quadder, Bernd, Braune, Renate, Skowasch, Dirk, Antoniou, Katerina, Bouros, Demosthenes, Gogali, Athena, Margaritopoulos, George A., Papakosta, Despoina, Papiris, Spyros A, Manali, Effrosyni D, Tzouvelekis, Argyris, Tzilas, Vasilios, Agrawal, Aditya, Kashyap, Surender, Tyagi, Vijay Narain, Unnati, Desai, Abedini, Atefeh, Phelan, Sean, Bargagli, Elena, Cinetto, Francesco, Confalonieri, Marco, Confalonieri, Paola, Harari, Sergio, Luppi, Fabrizio, Martone, Filippo, Palmieri, Anna, Rottoli, Paola, Salton, Francesco, Scarpa, Ricardo, Tana, Claudio, Tomassetti, Sara, Azuma, Arata, Inui, Naoki, Nakamura, Yutaro, Inoue, Yoshikazu, Tanino, Yoshinori, Muhammad, Noorul Afidza, van Beek, Frouke, den Boer, Lenze, Boomars, Karin, Bresser, Paul, Claessen, Laura, Elfferich, Marjon, Erckens, Roel, van Helmondt, Sanne, Karakaya, Bekir, Grutters, Jan C., Hoitsma, Elske, Illy, Károly, Jansen, Tim L, Kahlmann, Vivienne, Knoet, Chris, Korenromp, Ingrid, Lenssen, Ton, Maassen vd Brinkdrs, Karen, Mager, Johannes Jurgen, Marcellis, Rik, Miedema, Jelle, Möller, Trudeke, Moonen, Linda A.A., Moor, Karen, Mostard, Rémy L.M., Nienhuis, Willemien, Nossent, Esther, Paulus, Michelle, Ponds, Rudolf, Nossent, George, Quanjel, Marian, Rottenberg, Felix, van de Sande, Jonathan S.O., Segers, Ruud, Soechitram, Shalini D., van Staa, AnneLoes, Strookappe, Bert, Swaans, Martin, van den Toorn, Leon, van de Ven, Marjo J.T., Voortman, Mareye, Vorselaars, Adriane Dore Marie, Vente, Lenneke, Wijnen, Petal A., Wijsenbeek, Marlies, De Vries, Jolanda, van Zanten, Arthur, Durheim, Michael, Rasch-Halvorsen, Øystein, Tøndell, Anders, Dubaniewicz, Anna, Piotrowski, Wojciech, Roter, Bartlomiej, Robalo Cordeiro, Carlos, Alfaro, Tiago, Strambu, Irina, Vizel, Alexander, Cano, Rafael, Balcells, Eva, Castillo, Diego, Francesqui, Joel, Mañá, Juan, Molina, Maria, Ojanguren, Inigo, Rodriguez Portal, Jose Antonio, Sellares, Jacobo, Arkema, Elizabeth, Darlington, Pernilla, Eldhagen, Per, Elfström, Katinka, Grunewald, Johan, Kullberg, Susanna, Rivera, Natalia V, Rossides, Marios, Werner, Joanna, Dincer, Irem, Koç, Gülcan, Yucesan, Canan, Ozdemir Kumbasar, Ozlem, Birring, Surinder, Boyd, Jeanette, Casimo, Leo, Huntley, Christopher, Kouranos, Vasileios, Newton, Jaqui, Spencer, Lisa, Spears, Mark, Barreras, Paula, Cozier, Yvette, Crouser, Elliott, Harper, Logan, James, W. Ennis, Maier, Lisa, Liao, Shu-Yi, Morgenthau, Adam, Obi, Ogugua Ndili, Patel, Divya, Rivera, Frank, Rossman, Milton D., Sharp, Michelle, Sharma, Maggie, Schupp, Jonas C., Stern, Barney J., Sweiss, Nadera, Tavee, Jinny, Zank, Daniel, Gumeniuk, Galyna, Gavrysyuk, Volodymyr, Merenkova, Levgeniia, Drent, Marjolein, Russell, Anne-Marie, Saketkoo, Lesley Ann, Spagnolo, Paolo, Veltkamp, Marcel, and Wells, Athol U

Published
2024
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58. Smoking-related idiopathic interstitial pneumonia

Author

Flaherty, Kevin R, Fell, Charlene, Aubry, Marie-Christine, Brown, Kevin, Colby, Thomas, Costabel, Ulrich, Franks, Teri J, Gross, Barry H, Hansell, David M, Kazerooni, Ella, Kim, Dong Soon, King, Talmadge E, Kitachi, Masanori, Lynch, David, Myers, Jeff, Nagai, Sonoko, Nicholson, Andrew G, Poletti, Venerino, Raghu, Ganesh, Selman, Moises, Toews, Galen, Travis, William, Wells, Athol U, Vassallo, Robert, and Martinez, Fernando J

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Lung, Pneumonia & Influenza, Clinical Research, Tobacco Smoke and Health, Prevention, Tobacco, Infectious Diseases, Clinical Trials and Supportive Activities, Pneumonia, Respiratory, Adult, Aged, Carbon Monoxide, Female, Humans, Idiopathic Interstitial Pneumonias, International Cooperation, Male, Mental Recall, Mexico, Middle Aged, Models, Organizational, Prognosis, Pulmonary Medicine, Radiology, Republic of Korea, Retrospective Studies, Smoking, Tobacco Smoke Pollution, United Kingdom, United States, Medical and Health Sciences, Respiratory System, Cardiovascular medicine and haematology
Abstract

Cigarette smoking is a key factor in the development of numerous pulmonary diseases. An international group of clinicians, radiologists and pathologists evaluated patients with previously identified idiopathic interstitial pneumonia (IIP) to determine unique features of cigarette smoking. Phase 1 (derivation group) identified smoking-related features in patients with a history of smoking (n=41). Phase 2 (validation group) determined if these features correctly predicted the smoking status of IIP patients (n=100) to participants blinded to smoking history. Finally, the investigators sought to determine if a new smoking-related interstitial lung disease phenotype could be defined. Phase 1 suggested that preserved forced vital capacity with disproportionately reduced diffusing capacity of the lung for carbon monoxide, and various radiographic and histopathological findings were smoking-related features. In phase 2, the kappa coefficient among clinicians was 0.16 (95% CI 0.11-0.21), among the pathologists 0.36 (95% CI 0.32-0.40) and among the radiologists 0.43 (95% CI 0.35-0.52) for smoking-related features. Eight of the 100 cases were felt to represent a potential smoking-related interstitial lung disease. Smoking-related features of interstitial lung disease were identified in a minority of smokers and were not specific for smoking. This study is limited by its retrospective design, the potential for recall bias in smoking history and lack of information on second-hand smoke exposure. Further research is needed to understand the relationship between smoking and interstitial lung disease.

Published
2014

59. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials.

Author

Saketkoo, Lesley Ann, Mittoo, Shikha, Huscher, Dörte, Khanna, Dinesh, Dellaripa, Paul F, Distler, Oliver, Flaherty, Kevin R, Frankel, Sid, Oddis, Chester V, Denton, Christopher P, Fischer, Aryeh, Kowal-Bielecka, Otylia M, LeSage, Daphne, Merkel, Peter A, Phillips, Kristine, Pittrow, David, Swigris, Jeffrey, Antoniou, Katerina, Baughman, Robert P, Castelino, Flavia V, Christmann, Romy B, Christopher-Stine, Lisa, Collard, Harold R, Cottin, Vincent, Danoff, Sonye, Highland, Kristin B, Hummers, Laura, Shah, Ami A, Kim, Dong Soon, Lynch, David A, Miller, Frederick W, Proudman, Susanna M, Richeldi, Luca, Ryu, Jay H, Sandorfi, Nora, Sarver, Catherine, Wells, Athol U, Strand, Vibeke, Matteson, Eric L, Brown, Kevin K, Seibold, James R, and CTD-ILD Special Interest Group

Subjects
CTD-ILD Special Interest Group, Humans, Lung Diseases, Interstitial, Connective Tissue Diseases, Registries, Consensus, International Cooperation, Societies, Medical, Congresses as Topic, Randomized Controlled Trials as Topic, Idiopathic Pulmonary Fibrosis, Connective tissue disease associated lung disease, Idiopathic pulmonary fibrosis, Interstitial Fibrosis, Rheumatoid lung disease, Systemic disease and lungs, Lung Diseases, Interstitial, Societies, Medical, Respiratory System, Clinical Sciences
Abstract

RationaleClinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities.MethodsThe Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology-a non-profit international organisation dedicated to consensus methodology in identification of outcome measures-conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF).ResultsA core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed.ConclusionIdentification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field.

Published
2014

60. High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study.

Author

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

Subjects
DELPHI method, SARCOIDOSIS, PHENOTYPES, LIKERT scale, PHYSICIANS
Abstract

One view of sarcoidosis is that the term covers many different diseases. However, no classification framework exists for the future exploration of pathogenetic pathways, genetic or trigger predilections, patterns of lung function impairment, or treatment separations, or for the development of diagnostic algorithms or relevant outcome measures. We aimed to establish agreement on high-resolution CT (HRCT) phenotypic separations in sarcoidosis to anchor future CT research through a multinational two-round Delphi consensus process. Delphi participants included members of the Fleischner Society and the World Association of Sarcoidosis and other Granulomatous Disorders, as well as members' nominees. 146 individuals (98 chest physicians, 48 thoracic radiologists) from 28 countries took part, 144 of whom completed both Delphi rounds. After rating of 35 Delphi statements on a five-point Likert scale, consensus was achieved for 22 (63%) statements. There was 97% agreement on the existence of distinct HRCT phenotypes, with seven HRCT phenotypes that were categorised by participants as non-fibrotic or likely to be fibrotic. The international consensus reached in this Delphi exercise justifies the formulation of a CT classification as a basis for the possible definition of separate diseases. Further refinement of phenotypes with rapidly achievable CT studies is now needed to underpin the development of a formal classification of sarcoidosis. [ABSTRACT FROM AUTHOR]

Published
2024
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61. Serum C‐reactive protein is associated with earlier mortality across different interstitial lung diseases.

Author

Stock, Carmel J. W., Bray, William G., Kouranos, Vasilis, Jacob, Joseph, Kokosi, Maria, George, Peter M., Chua, Felix, Wells, Athol U., Sestini, Piersante, and Renzoni, Elisabetta A.

Subjects
BLOOD proteins, C-reactive protein, INTERSTITIAL lung diseases, IDIOPATHIC pulmonary fibrosis, HYPERSENSITIVITY pneumonitis, SYSTEMIC scleroderma, CARDIOVASCULAR diseases
Abstract

Background and Objective: The acute‐phase protein C‐reactive protein (CRP) is known to be associated with poor outcomes in cancer and cardiovascular disease, but there is limited evidence of its prognostic implications in interstitial lung diseases (ILDs). We therefore set out to test whether baseline serum CRP levels are associated with mortality in four different ILDs. Methods: In this retrospective study, clinically measured CRP levels, as well as baseline demographics and lung function measures, were collected for ILD patients first presenting to the Royal Brompton Hospital between January 2010 and December 2019. Cox regression analysis was used to determine the relationship with 5‐year mortality. Results: Patients included in the study were: idiopathic pulmonary fibrosis (IPF) n = 422, fibrotic hypersensitivity pneumonitis (fHP) n = 233, rheumatoid arthritis associated ILD (RA‐ILD) n = 111 and Systemic Sclerosis associated ILD (SSc‐ILD) n = 86. Patients with a recent history of infection were excluded. Higher CRP levels were associated with shorter 5‐year survival in all four disease groups on both univariable analyses, and after adjusting for age, gender, smoking history, immunosuppressive therapy and baseline disease severity (IPF: HR (95% CI): 1.3 (1.1–1.5), p = 0.003, fHP: 1.5 (1.2–1.9), p = 0.001, RA‐ILD: 1.4 (1.1–1.84), p = 0.01 and SSc‐ILD: 2.7 (1.6–4.5), p < 0.001). Conclusion: Higher CRP levels are independently associated with reduced 5‐year survival in IPF, fHP, RA‐ILD and SSc‐ILD. [ABSTRACT FROM AUTHOR]

Published
2024
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62. Mortality surrogates in combined pulmonary fibrosis and emphysema

Author

Zhao, An, primary, Gudmundsson, Eyjolfur, additional, Mogulkoc, Nesrin, additional, van Moorsel, Coline, additional, Corte, Tamera J., additional, Vasudev, Pardeep, additional, Romei, Chiara, additional, Chapman, Robert, additional, Wallis, Tim J.M., additional, Denneny, Emma, additional, Goos, Tinne, additional, Savas, Recep, additional, Ahmed, Asia, additional, Brereton, Christopher J., additional, van Es, Hendrik W., additional, Jo, Helen, additional, De Liperi, Annalisa, additional, Duncan, Mark, additional, Pontoppidan, Katarina, additional, De Sadeleer, Laurens J., additional, van Beek, Frouke, additional, Barnett, Joseph, additional, Cross, Gary, additional, Procter, Alex, additional, Veltkamp, Marcel, additional, Hopkins, Peter, additional, Moodley, Yuben, additional, Taliani, Alessandro, additional, Taylor, Magali, additional, Verleden, Stijn, additional, Tavanti, Laura, additional, Vermant, Marie, additional, Nair, Arjun, additional, Stewart, Iain, additional, Janes, Sam M., additional, Young, Alexandra L., additional, Barber, David, additional, Alexander, Daniel C., additional, Porter, Joanna C., additional, Wells, Athol U., additional, Jones, Mark G., additional, Wuyts, Wim A., additional, and Jacob, Joseph, additional

Published
2023
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64. Pulmonary hemodynamics and transplant‐free survival in sarcoidosis‐associated pulmonary hypertension: Results from an international registry

Author

Gayen, Shameek K., primary, Baughman, Robert P., additional, Nathan, Steven D., additional, Wells, Athol U., additional, Kouranos, Vasilis, additional, Alhamad, Esam H., additional, Culver, Daniel A., additional, Barney, Joseph, additional, Carmoma, Eva M., additional, Cordova, Francis C., additional, Huitema, Marloes, additional, Scholand, Mary Beth, additional, Wijsenbeek, Marlies, additional, Ganesh, Sivagini, additional, Birring, Surinder S., additional, Price, Laura C., additional, Wort, Stephen J., additional, Shlobin, Oksana A., additional, and Gupta, Rohit, additional

Published
2023
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67. Real-world data from 24 UK centres for the use of nintedanib for progressive fibrosing interstitial lung disease

Author

Dixon, Giles, primary, Hague, Samuel, additional, Mulholland, Sarah, additional, Khin, Aye Myat Noe, additional, Thould, Hannah, additional, Connon, Roisin, additional, Minnis, Paul, additional, Murtagh, Eoin, additional, Khan, Fasihul, additional, Toor, Sameen, additional, Lawrence, Alexandra, additional, Naqvi, Marium, additional, West, Alex, additional, Coker, Robina, additional, Ward, Katie, additional, Yazbeck, Leda, additional, Hart, Simon, additional, Garfoot, Theresa, additional, Newman, Kate, additional, Rivera-Ortega, Pilar, additional, Stranks, Lachlan, additional, Beirne, Paul, additional, Bradley, Jessica, additional, Rowan, Catherine, additional, Agnew, Sarah, additional, Ahmad, Mahin, additional, Spencer, Lisa G, additional, Aigbirior, Joshua, additional, Fahim, Ahmed, additional, Wilson, Andrew M, additional, Butcher, Elizabeth, additional, Chong, Sy Giin, additional, Saini, Gauri, additional, Zulfikar, Sabrina, additional, Chua, Felix, additional, George, Peter M, additional, Kokosi, Maria, additional, Kouranos, Vasileios, additional, Molyneaux, Philip, additional, Renzoni, Elisabetta, additional, Vitri, Benedetta, additional, Wells, Athol U, additional, Nicol, Lisa M, additional, Bianchi, Stephen, additional, Kular, Raman, additional, Liu, Huajian, additional, John, Alexander, additional, Barth, Sarah, additional, Wickremasinghe, Melissa, additional, Forrest, Ian A, additional, Grimes, Ian, additional, Simpson, A. John, additional, Fletcher, Sophie V, additional, Jones, Mark G, additional, Kinsella, Emma, additional, Naftel, Jennifer, additional, Wood, Nicola, additional, Chalmers, Jodie, additional, Crawshaw, Anjali, additional, Crowley, Louise E, additional, Dosanjh, Davinder, additional, Huntley, Christopher C, additional, Walters, Gareth I, additional, Gatheral, Timothy, additional, Plum, Catherine, additional, Bikmalla, Shiva, additional, Muthusami, Raja, additional, Stone, Helen, additional, Rodrigues, Jonathan C L, additional, Tsaneva-Atanasova, Krasimira, additional, Scotton, Chris J, additional, Gibbons*, Michael A, additional, and Barratt*, Shaney L, additional

Published
2023
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70. Introduction

Author

Wuyts, Wim A., primary, Cottin, Vincent, additional, Spagnolo, Paolo, additional, and Wells, Athol U., additional

Published
2019
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72. Clinical Assessment of Lung Disease

Author

Keir, Gregory J., Silver, Richard M., Wells, Athol U., Varga, John, editor, Denton, Christopher P., editor, Wigley, Fredrick M., editor, Allanore, Yannick, editor, and Kuwana, Masataka, editor

Published
2017
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73. Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial

Author

Visca, Dina, Mori, Letizia, Tsipouri, Vicky, Fleming, Sharon, Firouzi, Ashi, Bonini, Matteo, Pavitt, Matthew J, Alfieri, Veronica, Canu, Sara, Bonifazi, Martina, Boccabella, Cristina, De Lauretis, Angelo, Stock, Carmel J W, Saunders, Peter, Montgomery, Andrew, Hogben, Charlotte, Stockford, Anna, Pittet, Margaux, Brown, Jo, Chua, Felix, George, Peter M, Molyneaux, Philip L, Margaritopoulos, Georgios A, Kokosi, Maria, Kouranos, Vasileios, Russell, Anne Marie, Birring, Surinder S, Chetta, Alfredo, Maher, Toby M, Cullinan, Paul, Hopkinson, Nicholas S, Banya, Winston, Whitty, Jennifer A, Adamali, Huzaifa, Spencer, Lisa G, Farquhar, Morag, Sestini, Piersante, Wells, Athol U, and Renzoni, Elisabetta A

Published
2018
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74. Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

Author

Baughman, Robert P., Shlobin, Oksana A., Wells, Athol U., Alhamad, Esam H., Culver, Daniel A., Barney, Joseph, Cordova, Francis C., Carmona, Eva M., Scholand, Mary Beth, Wijsenbeek, Marlies, Ganesh, Sivagini, Birring, Surinder S., Kouranos, Vasilis, O'Hare, Lanier, Baran, Joanne M., Cal, Joseph G., Lower, Elyse E., Engel, Peter J., and Nathan, Steven D.

Published
2018
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79. An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias

Author

Travis, William D, Costabel, Ulrich, Hansell, David M, King, Talmadge E, Lynch, David A, Nicholson, Andrew G, Ryerson, Christopher J, Ryu, Jay H, Selman, Moisés, Wells, Athol U, Behr, Jurgen, Bouros, Demosthenes, Brown, Kevin K, Colby, Thomas V, Collard, Harold R, Cordeiro, Carlos Robalo, Cottin, Vincent, Crestani, Bruno, Drent, Marjolein, Dudden, Rosalind F, Egan, Jim, Flaherty, Kevin, Hogaboam, Cory, Inoue, Yoshikazu, Johkoh, Takeshi, Kim, Dong Soon, Kitaichi, Masanori, Loyd, James, Martinez, Fernando J, Myers, Jeffrey, Protzko, Shandra, Raghu, Ganesh, Richeldi, Luca, Sverzellati, Nicola, Swigris, Jeffrey, and Valeyre, Dominique

Subjects
Pneumonia & Influenza, Rare Diseases, Pneumonia, Lung, Clinical Research, Respiratory, Diagnosis, Differential, Europe, Humans, Idiopathic Interstitial Pneumonias, Societies, Medical, United States, ATS/ERS Committee on Idiopathic Interstitial Pneumonias, Medical and Health Sciences, Respiratory System
Abstract

BackgroundIn 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs.PurposeThe objective of this statement is to update the 2002 ATS/ERS classification of IIPs.MethodsAn international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011.ResultsSubstantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment.ConclusionsThis update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.

Published
2013

80. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis

Author

Fingerlin, Tasha E, Murphy, Elissa, Zhang, Weiming, Peljto, Anna L, Brown, Kevin K, Steele, Mark P, Loyd, James E, Cosgrove, Gregory P, Lynch, David, Groshong, Steve, Collard, Harold R, Wolters, Paul J, Bradford, Williamson Z, Kossen, Karl, Seiwert, Scott D, du Bois, Roland M, Garcia, Christine Kim, Devine, Megan S, Gudmundsson, Gunnar, Isaksson, Helgi J, Kaminski, Naftali, Zhang, Yingze, Gibson, Kevin F, Lancaster, Lisa H, Cogan, Joy D, Mason, Wendi R, Maher, Toby M, Molyneaux, Philip L, Wells, Athol U, Moffatt, Miriam F, Selman, Moises, Pardo, Annie, Kim, Dong Soon, Crapo, James D, Make, Barry J, Regan, Elizabeth A, Walek, Dinesha S, Daniel, Jerry J, Kamatani, Yoichiro, Zelenika, Diana, Smith, Keith, McKean, David, Pedersen, Brent S, Talbert, Janet, Kidd, Raven N, Markin, Cheryl R, Beckman, Kenneth B, Lathrop, Mark, Schwarz, Marvin I, and Schwartz, David A

Subjects
Biological Sciences, Genetics, Prevention, Lung, Rare Diseases, Human Genome, Pneumonia, Pneumonia & Influenza, Clinical Research, 2.1 Biological and endogenous factors, Aetiology, Respiratory, Case-Control Studies, Chromosomes, Human, Gene Expression, Gene Frequency, Genetic Loci, Genetic Predisposition to Disease, Genome-Wide Association Study, Humans, Idiopathic Pulmonary Fibrosis, Polymorphism, Single Nucleotide, Sequence Analysis, DNA, Medical and Health Sciences, Developmental Biology, Agricultural biotechnology, Bioinformatics and computational biology
Abstract

We performed a genome-wide association study of non-Hispanic, white individuals with fibrotic idiopathic interstitial pneumonias (IIPs; n = 1,616) and controls (n = 4,683), with follow-up replication analyses in 876 cases and 1,890 controls. We confirmed association with TERT at 5p15, MUC5B at 11p15 and the 3q26 region near TERC, and we identified seven newly associated loci (Pmeta = 2.4 × 10(-8) to 1.1 × 10(-19)), including FAM13A (4q22), DSP (6p24), OBFC1 (10q24), ATP11A (13q34), DPP9 (19p13) and chromosomal regions 7q22 and 15q14-15. Our results suggest that genes involved in host defense, cell-cell adhesion and DNA repair contribute to risk of fibrotic IIPs.

Published
2013

81. An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study

Author

Maher, Toby M, Oballa, Eunice, Simpson, Juliet K, Porte, Joanne, Habgood, Anthony, Fahy, William A, Flynn, Aiden, Molyneaux, Philip L, Braybrooke, Rebecca, Divyateja, Hrushikesh, Parfrey, Helen, Rassl, Doris, Russell, Anne-Marie, Saini, Gauri, Renzoni, Elisabetta A, Duggan, Anne-Marie, Hubbard, Richard, Wells, Athol U, Lukey, Pauline T, Marshall, Richard P, and Jenkins, R Gisli

Published
2017
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84. Antacid therapy in idiopathic pulmonary fibrosis: more questions than answers?

Author

Albera, Carlo, Antoniou, Katerina M., Altinisik, Goksel, Bendstrup, Elisabeth, Bondue, Benjamin, Borie, Raphael, Brown, Kevin K., Camus, Philippe, Castillo, Diego, Collard, Harold R., Cottin, Vincent, Crimi, Nunzio, Ferrara, Giovanni, Fischer, Aryeh, Gauldie, Jack, Geiser, Thomas, Guenther, Andreas, Hambly, Nathan, Hansell, David M., Harari, Sergio, Jones, Mark G., Keane, Michael, Ley, Brett, Maher, Toby M., Molina-Molina, Maria, Palmucci, Stefano, Poletti, Venerino, Prasse, Antje, Rottoli, Paola, Spagnolo, Paolo, Sterclova, Martina, Torrisi, Sebastiano, Tsitoura, Eliza, Vasakova, Martina, Walsh, Simon L., Wijsenbeek, Marlies S., Wuyts, Wim A., Johannson, Kerri A, Strâmbu, Irina, Ravaglia, Claudia, Grutters, Jan C, Valenzuela, Claudia, Mogulkoc, Nesrin, Luppi, Fabrizio, Richeldi, Luca, Wells, Athol U, Vancheri, Carlo, and Kreuter, Michael

Published
2017
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86. Efficacy of simtuzumab versus placebo in patients with idiopathic pulmonary fibrosis: a randomised, double-blind, controlled, phase 2 trial

Author

Raghu, Ganesh, Brown, Kevin K, Collard, Harold R, Cottin, Vincent, Gibson, Kevin F, Kaner, Robert J, Lederer, David J, Martinez, Fernando J, Noble, Paul W, Song, Jin Woo, Wells, Athol U, Whelan, Timothy P M, Wuyts, Wim, Moreau, Emmanuel, Patterson, Scott D, Smith, Victoria, Bayly, Selina, Chien, Jason W, Gong, Qi, Zhang, Jenny J, and O'Riordan, Thomas G

Published
2017
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89. Reply to: Pharmacotherapy for lung cancer with comorbid interstitial pneumonia: limited evidence requires appropriate evaluation

Author

Funke-Chambour, Manuela, Kewalramani, Namrata Murli, Machahua, Carlos, Poletti, Venerino, Wells, Athol U, and Cadranel, Jacques

Subjects
Pulmonary and Respiratory Medicine, 610 Medicine & health
Abstract

The divergent views on lung cancer treatments in fibrosing lung patients reflect differences due to variable side-effect incidences in different countries and among ethnicities. International efforts are needed to better define treatment approaches.

Published
2022

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

Author

Kim, John S, Montesi, Sydney B, Adegunsoye, Ayodeji, Humphries, Stephen M, Salisbury, Margaret L, Hariri, Lida P, Kropski, Jonathan A, Richeldi, Luca, Wells, Athol U, Walsh, Simon, Jenkins, R Gisli, Rosas, Ivan, Noth, Imre, Hunninghake, Gary M, Martinez, Fernando J, Podolanczuk, Anna J, Richeldi, Luca (ORCID:0000-0001-8594-1448), Kim, John S, Montesi, Sydney B, Adegunsoye, Ayodeji, Humphries, Stephen M, Salisbury, Margaret L, Hariri, Lida P, Kropski, Jonathan A, Richeldi, Luca, Wells, Athol U, Walsh, Simon, Jenkins, R Gisli, Rosas, Ivan, Noth, Imre, Hunninghake, Gary M, Martinez, Fernando J, Podolanczuk, Anna J, and Richeldi, Luca (ORCID:0000-0001-8594-1448)

Abstract

NO

Published
2023

99. Pulmonary hemodynamics and transplant-free survival in sarcoidosis-associated pulmonary hypertension:Results from an international registry

Author

Gayen, Shameek K., Baughman, Robert P., Nathan, Steven D., Wells, Athol U., Kouranos, Vasilis, Alhamad, Esam H., Culver, Daniel A., Barney, Joseph, Carmoma, Eva M., Cordova, Francis C., Huitema, Marloes, Scholand, Mary Beth, Wijsenbeek, Marlies, Ganesh, Sivagini, Birring, Surinder S., Price, Laura C., Wort, Stephen J., Shlobin, Oksana A., Gupta, Rohit, Gayen, Shameek K., Baughman, Robert P., Nathan, Steven D., Wells, Athol U., Kouranos, Vasilis, Alhamad, Esam H., Culver, Daniel A., Barney, Joseph, Carmoma, Eva M., Cordova, Francis C., Huitema, Marloes, Scholand, Mary Beth, Wijsenbeek, Marlies, Ganesh, Sivagini, Birring, Surinder S., Price, Laura C., Wort, Stephen J., Shlobin, Oksana A., and Gupta, Rohit

Abstract

Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac index (CI) ≤ 2 L/min/m2. However, there is no clear definition denoting severity of sarcoidosis-associated PH (SAPH). We aimed to determine pulmonary hemodynamic cut-off values where transplant-free survival was worse among patients with SAPH. This was a retrospective cohort analysis of the Registry of SAPH database focusing on pulmonary hemodynamic predictors of transplant-free survival among patients with precapillary SAPH. Cox regression was performed to determine which pulmonary hemodynamic values predicted death or lung transplantation. Kaplan−Meier survival analysis was performed on statistically significant predictors to determine pulmonary hemodynamic cut-off values where transplant-free survival was decreased. Decreased transplant-free survival occurred among SAPH patients with mPAP ≥ 40 mmHg and SAPH patients with pulmonary vascular resistance (PVR) ≥ 5 Woods units (WU). Transplant-free survival was not decreased in patients who fulfilled prior criteria of severe PH in chronic lung disease. We identified new cut-offs with decreased transplant-free survival in the SAPH population. Neither cut-off of mPAP ≥ 40 mmHg nor PVR ≥ 5 WU has previously been shown to be associated with decreased transplant-free survival in SAPH. These values could suggest a new definition of severe SAPH. Our PVR findings are in line with the most recent European Society of Cardiology/European Respiratory Society guideline definition of severe PH in chronic lung disease.

Published
2023

100. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study

Author

Walsh, Simon L F, Wells, Athol U, Desai, Sujal R, Poletti, Venerino, Piciucchi, Sara, Dubini, Alessandra, Nunes, Hilario, Valeyre, Dominique, Brillet, Pierre Y, Kambouchner, Marianne, Morais, António, Pereira, José M, Moura, Conceição Souto, Grutters, Jan C, van den Heuvel, Daniel A, van Es, Hendrik W, van Oosterhout, Matthijs F, Seldenrijk, Cornelis A, Bendstrup, Elisabeth, Rasmussen, Finn, Madsen, Line B, Gooptu, Bibek, Pomplun, Sabine, Taniguchi, Hiroyuki, Fukuoka, Junya, Johkoh, Takeshi, Nicholson, Andrew G, Sayer, Charlie, Edmunds, Lilian, Jacob, Joseph, Kokosi, Maria A, Myers, Jeffrey L, Flaherty, Kevin R, and Hansell, David M

Published
2016
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