Guidance for chest-CT in children and adults with cystic fibrosis: A European perspective

Michael Fayon¹, Kate Hill², Michael Waldron³, Barbara Messore⁴, Luca Riberi⁴, Marcus Svedberg⁵, Elise Lammertyn⁶, Stojka Fustik⁷, Andrea Gramegna⁸, Mirjam Stahl⁹, Anna Kerpel-Fronius¹⁰, Maurizio Balbi¹¹, Pierluigi Ciet¹², Guillaume Chassagnon¹³, Cinzia Ferrero¹⁴, Pierre-Régis Burgel¹⁵, Sivagurunathan Sutharsan¹⁶, Marcel Opitz¹⁷, Eleni-Rosalina Andrinopoulou¹⁸, Gael Dournes¹⁹, Michael Maher³, Jamie Duckers²⁰, Harm Tiddens²¹, Isabelle Sermet²²

Affiliations

¹ CHU Bordeaux, Département de Pédiatrie, CIC-P INSERM 1401 & Université de Bordeaux, Centre de Recherche Cardio-thoracique de Bordeaux, INSERM U1045, F-33000, Bordeaux, France. Electronic address: michael.fayon@chu-bordeaux.fr.
² European Cystic Fibrosis Society, Karup, Denmark; Northern Ireland Clinical Research Facility, The Wellcome-Wolfson Institute of Experimental Medicine, Queen's University, Lisburn Road, Belfast, Northern Ireland, UK. Electronic address: kate.hill@qub.ac.uk.
³ Cork Centre for Cystic Fibrosis, Cork University Hospital, University College Cork, Cork, Ireland; HRB Clinical Research Facility, University College Cork, Cork, Ireland; Department of Radiology, Cork University Hospital, Cork, Ireland.
⁴ AOU San Luigi Gonzaga, Adult CF Centre-Pulmonology Unit, Orbassano, Torino, Italy.
⁵ Department of Pediatrics, Institute of Clinical Science at The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Pediatrics, Queen Silvia's Children Hospital, Gothenburg, Sweden.
⁶ Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium.
⁷ Center for Cystic Fibrosis, University Children's Clinic, Skopje, North Macedonia.
⁸ Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
⁹ Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Berlin Institute of Health (BIH) at Charité - Universitätsmedizin Berlin, Berlin, Germany; German Center for Lung Research (DZL), Associated Partner Site, Berlin, Germany.
¹⁰ Department of Radiology, National Korányi Institute for Pulmonology, Budapest, Hungary.
¹¹ Radiology Unit, Department of Oncology, University of Turin, San Luigi Gonzaga Hospital, Orbassano, Italy.
¹² Department of Paediatrics, Division of Respiratory Medicine and Allergology, Sophia Children's Hospital, Erasmus MC, Rotterdam, the Netherlands; Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, the Netherlands.
¹³ Department of Radiology, Hôpital Cochin, AP-HP, Université Paris Cité, 27 Rue du Faubourg Saint-Jacques, 75014, Paris, France.
¹⁴ AOU Città della Salute e della Scienza di Torino, Regina Margherita Children's Hospital - Pediatric Pulmonology/Pediatric CF Centre, Torino, Italy.
¹⁵ Department of Respiratory Medicine and National Cystic Fibrosis Reference Centre, Groupe Hospitalier Cochin-Hôtel Dieu, AP-HP, Université Paris Cité and Institut Cochin, Inserm U1016, 27 Rue du Faubourg Saint-Jacques, 75014, Paris, France.
¹⁶ Department of Pulmonary Medicine, University Hospital Essen - Ruhrlandklinik, Adult Cystic Fibrosis Center, University of Duisburg-Essen, Essen, Germany.
¹⁷ Institute of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany.
¹⁸ Department of Biostatistics, Erasmus MC, Rotterdam, the Netherlands; Department of Epidemiology, Erasmus MC, Rotterdam, the Netherlands.
¹⁹ Univ. Bordeaux, INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, U1045, CIC 1401, Pessac, F-33600, France.
²⁰ All Wales Adult CF Centre, Cardiff and Vale University Health Board, Cardiff, UK.
²¹ Thirona, Nijmegen, the Netherlands.
²² Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Maladies Rares, Hôpital Necker Enfants Malades, Paris, 75015, France; INSERM U1151, Institut Necker Enfants Malades, Université Paris Cité, Paris, 75743, France; European Reference Network-Lung, Frankfurt, Germany.

PMID: 40189162
DOI: 10.1016/j.rmed.2025.108076

Free article

Review

Guidance for chest-CT in children and adults with cystic fibrosis: A European perspective

Michael Fayon et al. Respir Med. 2025 May.

Free article

. 2025 May:241:108076.

doi: 10.1016/j.rmed.2025.108076. Epub 2025 Apr 4.

Authors

Affiliations

¹ CHU Bordeaux, Département de Pédiatrie, CIC-P INSERM 1401 & Université de Bordeaux, Centre de Recherche Cardio-thoracique de Bordeaux, INSERM U1045, F-33000, Bordeaux, France. Electronic address: michael.fayon@chu-bordeaux.fr.
² European Cystic Fibrosis Society, Karup, Denmark; Northern Ireland Clinical Research Facility, The Wellcome-Wolfson Institute of Experimental Medicine, Queen's University, Lisburn Road, Belfast, Northern Ireland, UK. Electronic address: kate.hill@qub.ac.uk.
³ Cork Centre for Cystic Fibrosis, Cork University Hospital, University College Cork, Cork, Ireland; HRB Clinical Research Facility, University College Cork, Cork, Ireland; Department of Radiology, Cork University Hospital, Cork, Ireland.
⁴ AOU San Luigi Gonzaga, Adult CF Centre-Pulmonology Unit, Orbassano, Torino, Italy.
⁵ Department of Pediatrics, Institute of Clinical Science at The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Pediatrics, Queen Silvia's Children Hospital, Gothenburg, Sweden.
⁶ Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium.
⁷ Center for Cystic Fibrosis, University Children's Clinic, Skopje, North Macedonia.
⁸ Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
⁹ Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Berlin Institute of Health (BIH) at Charité - Universitätsmedizin Berlin, Berlin, Germany; German Center for Lung Research (DZL), Associated Partner Site, Berlin, Germany.
¹⁰ Department of Radiology, National Korányi Institute for Pulmonology, Budapest, Hungary.
¹¹ Radiology Unit, Department of Oncology, University of Turin, San Luigi Gonzaga Hospital, Orbassano, Italy.
¹² Department of Paediatrics, Division of Respiratory Medicine and Allergology, Sophia Children's Hospital, Erasmus MC, Rotterdam, the Netherlands; Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, the Netherlands.
¹³ Department of Radiology, Hôpital Cochin, AP-HP, Université Paris Cité, 27 Rue du Faubourg Saint-Jacques, 75014, Paris, France.
¹⁴ AOU Città della Salute e della Scienza di Torino, Regina Margherita Children's Hospital - Pediatric Pulmonology/Pediatric CF Centre, Torino, Italy.
¹⁵ Department of Respiratory Medicine and National Cystic Fibrosis Reference Centre, Groupe Hospitalier Cochin-Hôtel Dieu, AP-HP, Université Paris Cité and Institut Cochin, Inserm U1016, 27 Rue du Faubourg Saint-Jacques, 75014, Paris, France.
¹⁶ Department of Pulmonary Medicine, University Hospital Essen - Ruhrlandklinik, Adult Cystic Fibrosis Center, University of Duisburg-Essen, Essen, Germany.
¹⁷ Institute of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, Essen, Germany.
¹⁸ Department of Biostatistics, Erasmus MC, Rotterdam, the Netherlands; Department of Epidemiology, Erasmus MC, Rotterdam, the Netherlands.
¹⁹ Univ. Bordeaux, INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, U1045, CIC 1401, Pessac, F-33600, France.
²⁰ All Wales Adult CF Centre, Cardiff and Vale University Health Board, Cardiff, UK.
²¹ Thirona, Nijmegen, the Netherlands.
²² Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Maladies Rares, Hôpital Necker Enfants Malades, Paris, 75015, France; INSERM U1151, Institut Necker Enfants Malades, Université Paris Cité, Paris, 75743, France; European Reference Network-Lung, Frankfurt, Germany.

PMID: 40189162
DOI: 10.1016/j.rmed.2025.108076

Abstract

The European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN) herein proposes guidance for the use of chest CT-scans for the regular monitoring of lung disease in CF. Statements were completed in a 3-step process: the questions were identified via an anonymous online survey, followed by a comprehensive literature search, and a final Delphi process. The guidance recommends the use of ultra-low dose CT scans (effective radiation dose, 0.08 mSv; equivalent to 2 to 4 chest X-rays), tracking of patients' cumulative radiation and effective communication strategies using "de-medicalized" information for shared decision making. Chest CT scans (with lung volume monitoring) are not recommended systematically in both children and adults. Ultimate responsibility for justifying a chest CT scan lies with the individual professionals directly involved, the final decision being influenced by indications, costs, expertise, available material, resources and/or the patient's values, as well as possible impact on treatment modalities.

Keywords: Adult; CT-Scan; Child; Cystic fibrosis; Irradiation; Lung.

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Conflict of interest statement

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: I Sermet has received grants or contracts from the CFF grant to support paediatric research; has received institutional funding from Vertex Pharmaceuticals for academic research projects and is an investigator in Vertex Pharmaceuticals trials & consulting fees from Vertex and Eloxx for participation on Scientific Advisory Board. She is ECFS-CTN Standardisation Committee Chair & previous ECFS Board member & has received non-financial support for PTC Therapeutics for participation in Scientific Discussions. H Tiddens, Erasmus MC has received support from NIH, CFF, Sophia Foundation & NHMRC. The Erasmus MC holds a license agreement with Thirona and Resonance Health for the use of PRAGMA-CF. He has advised on Image analysis of dataset by Erasmus MC-Lung Analysis and Thirona & received payment or honoraria from Vertex for faculty for ADVANCE course, faculty for webinaron imaging. Erasmus MC and Thirona reimburse costs made for participating in relevant conferences as part of my work. He is Chair of imaging standardization committee ECFS-CTN & Emeritus Professor Erasmus MC; Founder, and former director Erasmus MC Lung Analysis Core laboratory & CMO Thirona. J Duckers has received payment or honoraria from Chiesi Pharmaceuticals, Insmed, Pfizer & Vertex & is a member of DSMB pharmaceuticals & Chair British Thoracic Society Specialist advisory group, Chair UK CF Registry. S Sutharsan has received grants from Vertex, Galapagos, Celtaxys, Proteostasis, Corbus and Insmed & consulting fees from Vertex, Boehringher Ingelheim and Insmed.P-R Burgel has received institutional support from GSK, Vertex, & consulting fees from Asta-Zeneca, Chiesi, GSK, Insmed, MSD, Vertex, Viatris and Zambon; support for attending meetings from Asta-Zeneca & Chiesi. G Chassagnon has received consulting fees from Gleamer SA, and payment or honoraria from Cheisi and Boehringher Ingelheim. P Ciet has received speaker fees from Vertex, Siemens Healthineers; grants or contracts from the Research Dutch Council, AIC Horizon Pathfinder and General Electrics Healthcare; payment or honoraria for lectures from Vertex, Siemens Healthineers & is Chair of the Cardiothoracic Taskforce ESPR & Member of the Research Commission ESPR. A-K Fronius is the President elect of the Hungarian Society of Radiologists & Member of the Board. M Stahl has received institutional support from the German Research Foundation (Deutsche Forschungsgemeinschaft, DFG); grants or constracts from Vertex Pharmaceuticals Independent RIA grant & for various clinical studies; payment or honoraria from Vertex Pharmaceuticals: honoraria for presentations including LEAD meetings; participation on DSMB for Vertex Pharmaceuticals: Advisory Board & is Chairman FGM, Secretary Group CF ERS & Treasurer GPP. A Gramegna has received payment or honoraria from Vertex and Chiesi; support for attending meetings from Foodar, Neupharma, Menarini & is ERS Group 10.3 Chair (Adult CF). Elise Lammetyn has received institutional support from Patient Centrics & Roche BeLux & is a member of the ECFS Patient Registry Scientific Committee. K Hill has received support for attending meetings from ECFS CTN, is a non-voting member of the ECFS-CTN Executive Committee and paid employee of the ECFS-CTN. M Fayon has a patient in the field of neonatal lung diseases: patent #FR3105004 & is President of the Pulmonology Society of the Indian Ocean. M Maher, M Opitz, C Ferrero, M Balbi, S Fustik, M Svedberg, B Messore, M Waldron, G Dournes & L Riberi have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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Guidance for chest-CT in children and adults with cystic fibrosis: A European perspective

Affiliations

Guidance for chest-CT in children and adults with cystic fibrosis: A European perspective

Authors

Affiliations

Abstract

Conflict of interest statement

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Medical