State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the"iMAging managEment of cySTic fibROsis" (MAESTRO) consortium

Pierluigi Ciet*, Silvia Bertolo, Mirco Ros, Rosaria Casciaro, Marco Cipolli, Stefano Colagrande, Stefano Costa, Valeria Galici, Andrea Gramegna, Cecilia Lanza, Francesca Lucca, Letizia Macconi, Fabio Majo, Antonella Paciaroni, Giuseppe Fabio Parisi, Francesca Rizzo, Ignazio Salamone, Teresa Santangelo, Luigia Scudeller, Luca SabaPaolo Tomà, Giovanni Morana

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

18 Citations (Scopus)
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Objective: Imaging represents an important noninvasive means to assess cystic fibrosis (CF) lung disease, which remains the main cause of morbidity and mortality in CF patients. While the development of new imaging techniques has revolutionised clinical practice, advances have posed diagnostic and monitoring challenges. The authors aim to summarise these challenges and make evidence-based recommendations regarding imaging assessment for both clinicians and radiologists. Study design: A committee of 21 experts in CF from the 10 largest specialist centres in Italy was convened, including a radiologist and a pulmonologist from each centre, with the overall aim of developing clear and actionable recommendations for lung imaging in CF. An a priori threshold of at least 80% of the votes was required for acceptance of each statement of recommendation. Results: After a systematic review of the relevant literature, the committee convened to evaluate 167 articles. Following five RAND conferences, consensus statements were developed by an executive subcommittee. The entire consensus committee voted and approved 28 main statements. Conclusions: There is a need for international guidelines regarding the appropriate timing and selection of imaging modality for patients with CF lung disease; timing and selection depends upon the clinical scenario, the patient's age, lung function and type of treatment. Despite its ubiquity, the use of the chest radiograph remains controversial. Both computed tomography and magnetic resonance imaging should be routinely used to monitor CF lung disease. Future studies should focus on imaging protocol harmonisation both for computed tomography and for magnetic resonance imaging. The introduction of artificial intelligence imaging analysis may further revolutionise clinical practice by providing fast and reliable quantitative outcomes to assess disease status. To date, there is no evidence supporting the use of lung ultrasound to monitor CF lung disease.

Original languageEnglish
Article number210173
JournalEuropean Respiratory Review
Issue number163
Publication statusPublished - 31 Mar 2022

Bibliographical note

Support statement: This work was supported by an unrestricted educational grant from Chiesi Pharmaceuticals, Parma, Italy and Vertex Pharmaceuticals, Boston, USA.

Publisher Copyright: © The authors 2022.


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