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

Sujal R Desai¹, Nishanth Sivarasan², Kerri A Johannson³, Peter M George⁴, Daniel A Culver⁵, Anand Devaraj⁶, David A Lynch⁷, David Milne⁸, Elisabetta Renzoni⁴, Hilario Nunes⁹, Nicola Sverzellati¹⁰, Paolo Spagnolo¹¹, Robert P Baughman¹², Ruchi Yadav¹³, Sara Piciucchi¹⁴, Simon L F Walsh¹⁵, Vasileios Kouranos⁴, Athol U Wells⁴; Sarcoid Delphi Group

Collaborators, Affiliations

Collaborators

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

Affiliations

¹ Department of Radiology, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK. Electronic address: s.desai@rbht.nhs.uk.
² Department of Radiology, Royal Brompton Hospital, London, UK.
³ Department of Medicine, University of Calgary, Calgary, AB, Canada.
⁴ Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK.
⁵ Department of Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, USA.
⁶ Department of Radiology, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK.
⁷ Department of Radiology, National Jewish Health, Denver, CO, USA.
⁸ Department of Radiology, Auckland City Hospital, Auckland, New Zealand.
⁹ Service de Pneumologie, Hôpital Avicenne, Université Sorbonne Paris Nord, Paris, France.
¹⁰ Section of Diagnostic Imaging, University of Parma, Parma, Italy.
¹¹ Section of Respiratory Diseases, University of Padova, Padova, Italy.
¹² Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA.
¹³ Imaging Institute, Cleveland Clinic, Cleveland, OH, USA.
¹⁴ Department of Radiology, GB Morgagni Hospital, Forlì, Italy.
¹⁵ National Heart and Lung Institute, Imperial College London, London, UK.

PMID: 38104579
DOI: 10.1016/S2213-2600(23)00267-9

Review

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

Sujal R Desai et al. Lancet Respir Med. 2024 May.

. 2024 May;12(5):409-418.

doi: 10.1016/S2213-2600(23)00267-9. Epub 2023 Dec 14.

Authors

Collaborators

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

Affiliations

¹ Department of Radiology, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK. Electronic address: s.desai@rbht.nhs.uk.
² Department of Radiology, Royal Brompton Hospital, London, UK.
³ Department of Medicine, University of Calgary, Calgary, AB, Canada.
⁴ Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK.
⁵ Department of Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, USA.
⁶ Department of Radiology, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK; Margaret Turner Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK.
⁷ Department of Radiology, National Jewish Health, Denver, CO, USA.
⁸ Department of Radiology, Auckland City Hospital, Auckland, New Zealand.
⁹ Service de Pneumologie, Hôpital Avicenne, Université Sorbonne Paris Nord, Paris, France.
¹⁰ Section of Diagnostic Imaging, University of Parma, Parma, Italy.
¹¹ Section of Respiratory Diseases, University of Padova, Padova, Italy.
¹² Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA.
¹³ Imaging Institute, Cleveland Clinic, Cleveland, OH, USA.
¹⁴ Department of Radiology, GB Morgagni Hospital, Forlì, Italy.
¹⁵ National Heart and Lung Institute, Imperial College London, London, UK.

PMID: 38104579
DOI: 10.1016/S2213-2600(23)00267-9

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.

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Conflict of interest statement

Declaration of interests SRD has received consulting fees for participation as an external expert steering committee member for a phase 3b/4 trial of trastuzumab deruxtecan in stage IV breast cancer from AstraZeneca and honoraria for travel expenses and for tutoring on radiology masterclasses in interstitial lung disease (ILD) from Boehringer Ingelheim; he is a co-founder and clinical director of, and holds shares in, DMC Radiology Reporting (an outsourcing, teleradiology company with no research or commercial interest in sarcoidosis). NSi has received honoraria for lectures from the World Association of Sarcoidosis and other Granulomatous Disorders (WASOG), Associazione Polo Italia-Cina, and Boehringer Ingelheim. KAJ has received grants from Three Lakes Foundation, the Chest Foundation, the University of Calgary Cumming School of Medicine, and the University Hospital Foundation (affiliated with the University of Alberta), consulting fees from Boehringer Ingelheim, Hoffman-La Roche, Pliant Therapeutics, Three Lakes Foundation, and Thyron SAB, and honoraria for lectures from Boehringer Ingelheim and Hoffman-La Roche; she has participated on the data safety monitoring board (DSMB) for the Ambulatory Oxygen for Treatment of Exertional Hypoxaemia in Pulmonary Fibrosis (PFOX) trial (no payment received). PMG has received grants from Boehringer Ingelheim, speaker's fees from Boehringer Ingelheim, Roche, Teva, Cipla, and Brainomix, and travel support from Boehringer Ingelheim and Roche; he is a medical director of, and holds stock options in, Brainomix (a company that develops artificial intelligence-powered imaging technologies unrelated to the submitted work). DAC has received grants from Boehringer Ingelheim, the Ann Theodore Foundation, the Foundation for Sarcoidosis Research, and aTyr, consulting fees from Kinevant and CSL Behring, and travel support from Roche. AD has received consulting fees from Boehringer Ingelheim, Roche, Brainomix, and Vicore; he is a medical director of, and holds stock options in, Brainomix. DAL has received consulting fees from Calyx and has participated on the advisory board for Boehringer Ingelheim. ER has received grants from Boehringer Ingelheim, honoraria for lectures from Boehringer Ingelheim and Chiesi, and travel support from Boehringer Ingelheim; she has participated on advisory boards for Boehringer Ingelheim and Roche. PS has received grants from PPM Services, Boehringer Ingelheim, and Roche, consulting fees from Novartis, Behring, PPM Services, Pieris, and GlycoCore, honoraria for lectures from Chiesi, Boehringer Ingelheim, and Menarini, support for attending meetings from PPM Services and Boehringer Ingelheim, and fees for participation on a DSMB or advisory board from Galapagos, Pieris, Boehringer Ingelheim, and Novartis, all outside of the submitted work. RPB has received grants from aTyr, Mallinckroft, Bellephron, Genentech, Actelion, and the Foundation for Sarcoidosis Research, consulting fees from aTyr, Kinevent, Bellephron, and the Millikin Center, and speaker's fees for lectures on sarcoidosis and other ILDs from Boehringer Ingelheim, Mallinckrodt, and United Therapeutics. SLFW has received grants from Boehringer Ingelheim and Galapagos, consulting fees from Boehringer Ingelheim, Roche, Galapagos, the Open Source Imaging Consortium, Puretech, Pliant, Oncorendi Therapeutics, and FLUIDDA, and honoraria for lectures and for developing educational material from Boehringer Ingelheim; he has participated on advisory boards for Boehringer Ingelheim and Roche. VK has received speaker's fees from Boehringer Ingelheim and Novartis. AUW has received consulting fees from Roche and Veracyte, and honoraria for lectures and presentations from Boehringer Ingelheim, Roche, and Veracyte; he is the president of the WASOG. All other authors declare no competing interests.

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

Collaborators

Affiliations

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

Authors

Collaborators

Affiliations

Abstract

Conflict of interest statement

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