Figures
- FIGURE 1
Flowchart of article selection.
Tables
- TABLE 1
The three clinical scenarios
Clinical scenario Type of patient Statements First diagnosis Asymptomatic 1.1–1.4 Symptomatic Follow-up Stable 2.1–2.18 Declining Improving (treatment with cystic fibrosis transmembrane conductance regulator modulators) 2.10 and 2.12 Pulmonary exacerbation Diagnosis 3.1–3.6 Monitoring treatment response - TABLE 2
First diagnosis
Statement number Statement Type of statement Strength of recommendation Quality of evidence Type of patient Most relevant supporting articles 1.1 In 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. Recommendation Grade B Moderate Asymptomatic and symptomatic [10, 13, 29, 30] 1.2 Low-dose CT is feasible both in uncooperative and cooperative patients. Recommendation Grade A High Asymptomatic and symptomatic [31–36] 1.3 Although 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. Recommendation Grade B Moderate Asymptomatic and symptomatic [7–9, 28, 33, 37–39] 1.4 CT dose should be as low as reasonably achievable without affecting the diagnostic quality of the image. Recommendation Grade A High Asymptomatic and symptomatic [14, 40–44] CT: computed tomography; MRI: magnetic resonance imaging.
- TABLE 3
Follow-up
Statement number Statement Type of statement Strength of recommendation Quality of evidence Type of patient Most relevant supporting articles 2.1 Limited 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. Recommendation Grade I Low Stable and declining [45, 46] 2.2 Use 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.Recommendation Grade C Moderate Stable and declining [47–52] 2.3 There 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 fact NA Low Stable [6, 13, 29, 53–56] 2.4 Current best clinical imaging practice in several CF centres is performing CT biennially (i.e. 1 every 2 years). Best practice Grade A Moderate Stable [14, 15] 2.5 State-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.Recommendation Grade A High Stable [14, 29, 35, 36, 40–44] 2.6 CT can better detect lung disease progression than standard pulmonary function tests (i.e. FEV1) both in cooperative and uncooperative patients, irrespective of disease severity. Recommendation Grade B High Stable and declining [5, 10, 12, 53–66] 2.7 CT is complementary to lung clearance index in the detection of disease progression or improvement by clinical intervention. Statement of fact NA High Stable and declining [67–74] 2.8 CT provides relevant information possibly capable of modifying disease trajectory, patient management and follow-up, both in uncooperative and cooperative patients. Statement of fact NA Moderate Stable and declining [5, 10–12, 48, 75, 76] 2.9 The 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. Recommendation Grade A Moderate Stable and declining [60, 65, 77–94] 2.10 Artificial 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 fact NA Moderate Stable, declining and improving [60, 65, 77–94] 2.11 CT 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 fact NA Moderate Declining [34, 48, 53, 60, 63, 92, 95–102] 2.12 Despite 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 fact NA Moderate Stable, declining and improving [35, 36, 81, 101, 103–105] 2.13 Despite 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 fact NA High Stable and declining [8, 9, 106–118] 2.14 MRI provides information about ventilation, inflammation, perfusion and structure (VIPS-MRI) in a single examination that is difficult to obtain with CT. Statement of fact Grade B Moderate Stable and declining [8, 9, 33, 119–133] 2.15 MRI 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 fact Grade B Moderate Stable and declining [118, 131, 134–138] 2.16 The 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 fact NA Moderate Stable and declining [115, 128, 139–142] 2.17 Longitudinal studies are needed to compare the sensitivity of CT and MRI in tracking disease progression. Statement of fact NA Moderate Stable and declining [8, 116, 143] 2.18 The 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 fact NA High Stable 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 number Statement Type of statement Strength of recommendation Quality of evidence Type of patient Most relevant supporting articles 3.1 CR may not help with the detection of pulmonary exacerbation, especially in CF patients with more severe disease. Recommendation Grade D Low Diagnosis [149] 3.2 CT 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 fact NA High Diagnosis [89, 95, 96, 150–156] 3.3 CT 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. Recommendation Grade C Low Diagnosis and monitoring treatment response [55, 65, 89, 95, 96, 150–154, 157] 3.4 Routine 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. Recommendation Grade D Moderate Monitoring treatment response [14, 29, 35, 36, 40–44, 158] 3.5 MRI 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. Recommendation Grade A High Diagnosis and monitoring treatment response [118, 119, 130, 136, 159] 3.6 MRI can be used as a clinically relevant outcome predictor for pulmonary exacerbations in cooperative, declining CF patients. Statement of fact NA High Diagnosis 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.
Supplementary Material
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Supplementary material ERR-0173-2021.SUPPLEMENT
Vol 31 Issue 163
Table of Contents
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
