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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 Saba, Paolo Tomà, Giovanni Morana
European Respiratory Review 2022 31: 210173; DOI: 10.1183/16000617.0173-2021
Pierluigi Ciet
1Radiology and Nuclear Medicine Dept, Erasmus MC, Rotterdam, The Netherlands
2Pediatric Pulmonology and Allergology Dept, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands
3Depts of Radiology and Medical Science, University of Cagliari, Cagliari, Italy
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  • For correspondence: p.ciet@erasmusmc.nl
Silvia Bertolo
4Radiology Dept, Ca'Foncello S. Maria Hospital, Treviso, Italy
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Mirco Ros
5Dept of Pediatrics, Ca'Foncello S. Maria Hospital, Treviso, Italy
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Rosaria Casciaro
6Dept of Pediatrics, IRCCS Institute “Giannina Gaslini”, Cystic Fibrosis Centre, Genoa, Italy
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Marco Cipolli
7Regional Reference Cystic Fibrosis center, University hospital of Verona, Verona, Italy
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Stefano Colagrande
8Dept of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit n. 2, University of Florence- Careggi Hospital, Florence, Italy
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  • ORCID record for Stefano Colagrande
Stefano Costa
9Dept of Pediatrics, Gaetano Martino Hospital, Messina, Italy
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Valeria Galici
10Cystic Fibrosis Centre, Dept of Paediatric Medicine, Anna Meyer Children's University Hospital, Florence, Italy
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Andrea Gramegna
11Respiratory Disease and Adult Cystic Fibrosis Centre, Internal Medicine Dept, IRCCS Ca' Granda, Milan, Italy
12Dept of Pathophysiology and Transplantation, University of Milan, Milan, Italy
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Cecilia Lanza
13Radiology Dept, University Hospital Ospedali Riuniti, Ancona, Italy
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Francesca Lucca
7Regional Reference Cystic Fibrosis center, University hospital of Verona, Verona, Italy
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Letizia Macconi
14Radiology Dept, Tuscany Reference Cystic Fibrosis Centre, Meyer Children's Hospital, Florence, Italy
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Fabio Majo
15Dept of Pediatrics, IRCCS Bambino Gesù Children's Hospital, Rome, Italy
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Antonella Paciaroni
16Radiology Dept, “Santa Maria del Carmine” Hospital, Rovereto, Italy
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Giuseppe Fabio Parisi
17Pediatric Pulmonology Unit, Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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  • ORCID record for Giuseppe Fabio Parisi
Francesca Rizzo
18Radiology Dept, IRCCS Institute “Giannina Gaslini”, Cystic Fibrosis Center, Genoa, Italy
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Ignazio Salamone
19Dept of Radiology, Gaetano Martino Hospital, Messina, Italy
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Teresa Santangelo
20Dept of Radiology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy
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Luigia Scudeller
21Clinical Epidemiology, IRCCS Azienda Ospedaliera Universitaria di Bologna, Bologna, Italy
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Luca Saba
3Depts of Radiology and Medical Science, University of Cagliari, Cagliari, Italy
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Paolo Tomà
20Dept of Radiology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy
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Giovanni Morana
4Radiology Dept, Ca'Foncello S. Maria Hospital, Treviso, Italy
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  • ORCID record for Giovanni Morana
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  • TABLE 1

    The three clinical scenarios

    Clinical scenarioType of patientStatements
    First diagnosisAsymptomatic1.1–1.4
    Symptomatic
    Follow-upStable2.1–2.18
    Declining
    Improving (treatment with cystic fibrosis transmembrane conductance regulator modulators)2.10 and 2.12
    Pulmonary exacerbationDiagnosis3.1–3.6
    Monitoring treatment response
  • TABLE 2

    First diagnosis

    Statement numberStatementType of statementStrength of recommendationQuality of evidenceType of patientMost relevant supporting articles
    1.1In infants diagnosed via newborn screening, CT can be used as a sensitive tool to detect early disease, tailor treatment, and monitor disease progression both in symptomatic and asymptomatic patients.RecommendationGrade BModerateAsymptomatic and symptomatic[10, 13, 29, 30]
    1.2Low-dose CT is feasible both in uncooperative and cooperative patients.RecommendationGrade AHighAsymptomatic and symptomatic[31–36]
    1.3Although MRI is more feasible in cooperative patients, it can be performed in uncooperative patients with or without moderate sedation/general anaesthesia according to the patient's age and mental status.RecommendationGrade BModerateAsymptomatic and symptomatic[7–9, 28, 33, 37–39]
    1.4CT dose should be as low as reasonably achievable without affecting the diagnostic quality of the image.RecommendationGrade AHighAsymptomatic and symptomatic[14, 40–44]

    CT: computed tomography; MRI: magnetic resonance imaging.

    • TABLE 3

      Follow-up

      Statement numberStatementType of statementStrength of recommendationQuality of evidenceType of patientMost relevant supporting articles
      2.1Limited data are available about the use of LUS to monitor lung status or to evaluate pulmonary exacerbation. Preliminary results show good relation between LUS and CT to assess structural changes.RecommendationGrade ILowStable and declining[45, 46]
      2.2Use of CR scoring systems can improve sensitivity in monitoring CF lung disease. However, their routine use in clinical practice is cumbersome, due to high inter-observer variability.
      Fully automated CR scoring systems based on artificial intelligence algorithms may overcome this limitation increasing the sensitivity of CR in detecting disease progression.
      RecommendationGrade CModerateStable and declining[47–52]
      2.3There is little evidence in the literature about the optimal timing of CT monitoring. There is a need for international guidelines to schedule CT surveillance in patients with CF lung disease.Statement of factNALowStable[6, 13, 29, 53–56]
      2.4Current best clinical imaging practice in several CF centres is performing CT biennially (i.e. 1 every 2 years).Best practiceGrade AModerateStable[14, 15]
      2.5State-of-the-art reviews of risk related to CT radiation exposure highlight a reasonably low risk of cumulative cancer in children using biennial low dose CT.
      CT protocol harmonisation between CF centres should be promoted to comply with the “as low as reasonably achievable” (ALARA) concept.
      RecommendationGrade AHighStable[14, 29, 35, 36, 40–44]
      2.6CT can better detect lung disease progression than standard pulmonary function tests (i.e. FEV1) both in cooperative and uncooperative patients, irrespective of disease severity.RecommendationGrade BHighStable and declining[5, 10, 12, 53–66]
      2.7CT is complementary to lung clearance index in the detection of disease progression or improvement by clinical intervention.Statement of factNAHighStable and declining[67–74]
      2.8CT provides relevant information possibly capable of modifying disease trajectory, patient management and follow-up, both in uncooperative and cooperative patients.Statement of factNAModerateStable and declining[5, 10–12, 48, 75, 76]
      2.9The use of appropriate scoring systems for CT increases its sensitivity in tracking changes in symptomatic, and asymptomatic, early lung disease. Therefore, their use is recommended to standardise interpretation of CT data according to CF centre expertise and capacity.RecommendationGrade AModerateStable and declining[60, 65, 77–94]
      2.10Artificial intelligence-based scoring system and segmentation tools for CF imaging allows a fully automated volumetric quantification of CF-related abnormalities over an entire lung. These novel scoring systems, when further validated, could provide a robust disease outcome in the era of effective CFTR modulator therapy.Statement of factNAModerateStable, declining and improving[60, 65, 77–94]
      2.11CT scans performed in infants and young children with symptoms is a potential clinical trial outcome measure for novel treatments in this age group.Statement of factNAModerateDeclining[34, 48, 53, 60, 63, 92, 95–102]
      2.12Despite improvement in clinical, lung function, and imaging outcomes in patients undertaking CFTR modulator therapy, no deviation from the usual imaging follow-up scheme should be advised, because there is no evidence in the current literature about long-term benefit of these agents.Statement of factNAModerateStable, declining and improving[35, 36, 81, 101, 103–105]
      2.13Despite conventional MRI sequences having lower sensitivity than CT in the assessment of disease extent, state-of-the-art MRI (e.g. UTE sequence) shows comparable results to CT and provides a convenient, noninvasive, and non-ionising assessment of disease progression in cooperative patients. The beneficial absence of radiation is particularly important with respect to the need for frequent follow-up examinations and the increasing life span of CF patients.Statement of factNAHighStable and declining[8, 9, 106–118]
      2.14MRI provides information about ventilation, inflammation, perfusion and structure (VIPS-MRI) in a single examination that is difficult to obtain with CT.Statement of factGrade BModerateStable and declining[8, 9, 33, 119–133]
      2.15MRI is a noninvasive, radiation-free endpoint to identify potentially reversible abnormalities (e.g. mucus plugging and lung hypoperfusion) in early phase clinical trials testing novel therapeutics in symptomatic, cooperative, patients with CF.Statement of factGrade BModerateStable and declining[118, 131, 134–138]
      2.16The MRI scoring system is a promising tool to predict the loss of lung function in CF patients and can serve as a clinically relevant outcome predictor for pulmonary manifestations in CF.Statement of factNAModerateStable and declining[115, 128, 139–142]
      2.17Longitudinal studies are needed to compare the sensitivity of CT and MRI in tracking disease progression.Statement of factNAModerateStable and declining[8, 116, 143]
      2.18The use of MRI in clinical practice is hampered by its higher cost than CT; the need for state-of-the-art MR systems; the occasional need for moderate sedation/general anaesthesia in uncooperative children; nonuniformity of MR protocol; as well as substantial image variability/capability between MR brands.Statement of factNAHighStable and declining[7, 8, 34, 37, 144–148]

      CF: cystic fibrosis; CFTR: cystic fibrosis transmembrane conductance regulator; CR: chest radiograph; CT: computed tomography; FEV1: forced expiratory volume in 1 s; LUS: lung ultrasound; MR: MRI: magnetic resonance; MRI: magnetic resonance imaging; NA: not available; UTE: ultrashort echo.

      • TABLE 4

        Pulmonary exacerbations

        Statement numberStatementType of statementStrength of recommendationQuality of evidenceType of patientMost relevant supporting articles
        3.1CR may not help with the detection of pulmonary exacerbation, especially in CF patients with more severe disease.RecommendationGrade DLowDiagnosis[149]
        3.2CT can detect acute structural lung abnormalities during and after pulmonary exacerbation (e.g. increase of bronchial wall thickening and mucus plugging) in cooperative, and uncooperative, patients.Statement of factNAHighDiagnosis[89, 95, 96, 150–156]
        3.3CT can be used to define outcome measures of pulmonary damage in clinical trials and test therapeutic interventions in patients with persistent respiratory symptoms and reduced lung function despite appropriate therapy.RecommendationGrade CLowDiagnosis and monitoring treatment response[55, 65, 89, 95, 96, 150–154, 157]
        3.4Routine use of CT for short term follow-up during pulmonary exacerbation is not recommended due to the risk of high cumulative radiation dose. Clinicians should consider the risk/benefit ratio related to dose when prescribing CT in pulmonary exacerbation.RecommendationGrade DModerateMonitoring treatment response[14, 29, 35, 36, 40–44, 158]
        3.5MRI can be used as a surrogate marker for disease severity and response to treatment during short-term follow-up of symptomatic, or declining, cooperative CF patients. In uncooperative patients, the risk related to moderate sedation or anaesthesia needs to be considered.RecommendationGrade AHighDiagnosis and monitoring treatment response[118, 119, 130, 136, 159]
        3.6MRI can be used as a clinically relevant outcome predictor for pulmonary exacerbations in cooperative, declining CF patients.Statement of factNAHighDiagnosis and monitoring treatment response[106, 116, 119, 130, 139]

        CF: cystic fibrosis; CR: chest radiograph; CT: computed tomography; MRI: magnetic resonance imaging; NA: not available.

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        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 Saba, Paolo Tomà, Giovanni Morana
        European Respiratory Review Mar 2022, 31 (163) 210173; DOI: 10.1183/16000617.0173-2021

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        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 Saba, Paolo Tomà, Giovanni Morana
        European Respiratory Review Mar 2022, 31 (163) 210173; DOI: 10.1183/16000617.0173-2021
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