• chronic obstructive pulmonary disease (COPD)
• emphysema
• long non-coding interleukin-7 receptor α-subunit gene (lnc-IL7R)
• machine learning
• percentage of low attenuation area (LAA%)

• 112-2634-F002-003 National Science and Technology Council of Taiwan
• 113-2222-E-038-003 National Science and Technology Council of Taiwan
• TMU113-AE1-B03 Taipei Medical University
• 112TMU-SHH-05 Taipei Medical University

To assess airway and lung parenchymal damage noninvasively in cystic fibrosis (CF), chest MRI has been historically out of the scope of routine clinical imaging because of technical difficulties such as low proton density and respiratory and cardiac motion. However, technological breakthroughs have emerged that dramatically improve lung MRI quality (including signal-to-noise ratio, resolution, speed, and contrast). At the same time, novel treatments have changed the landscape of CF clinical care. In this contemporary context, there is now consensus that lung MRI can be used clinically to assess CF in a radiation-free manner and to enable quantification of lung disease severity. MRI can now achieve three-dimensional, high-resolution morphologic imaging, and beyond this morphologic information, MRI may offer the ability to sensitively differentiate active inflammation vs scarring tissue. MRI could also characterize various forms of inflammation for early guidance of treatment. Moreover, functional information from MRI can be used to assess regional, small-airway disease with sensitivity to detect small changes even in patients with mild CF. Finally, automated quantification methods have emerged to support conventional visual analyses for more objective and reproducible assessment of disease severity. This article aims to review the most recent developments of lung MRI, with a focus on practical application and clinical value in CF, and the perspectives on how these modern techniques may converge and impact patient care soon.

• cystic fibrosis
• imaging
• lung
• magnetic resonance

Cystic fibrosis (CF) is the most common autosomal recessive disease of the Caucasian population worldwide, with respiratory disease remaining the most relevant source of morbidity and mortality. Computed tomography (CT) is frequently used for monitoring disease complications and progression. Over the last fifteen years there has been a six-fold increase in the use of CT, which has lead to a growing concern in relation to cumulative radiation exposure. The challenge to the medical profession is to identify dose reduction strategies that meet acceptable image quality, but fulfil the requirements of a diagnostic quality CT. Dose-optimisation, particularly in CT, is essential as it reduces the chances of patients receiving cumulative radiation doses in excess of 100 mSv, a dose deemed significant by the United Nations Scientific Committee on the Effects of Atomic Radiation. This review article explores the current trends in imaging in CF with particular emphasis on new developments in dose optimisation.

• Cystic fibrosis
• Dose optimization
• Computed tomography
• Dose
• Ionising radiation
• Kalydeco

• Bhalla-score
• Lung Clearance Index
• Mild Cystic Fibrosis lung disease
• Multiple Breath Washout
• Ultra-low-dose chest computed tomography

The first radiographic scoring system for pulmonary cystic fibrosis was presented in 1958. Since then a multitude of scoring systems for radiography and computed tomography (CT) have been presented, recently also for tomosynthesis and magnetic resonance imaging (MRI). The aim of the current review was to analyse and compare the plethora of scoring systems for cystic fibrosis, especially regarding which scoring components are considered most important.

Four scoring systems for chest radiography, one for tomosynthesis, eight for CT and one for MRI were compared regarding components evaluated and their terminology; the areas scored; scoring levels; the weighting of each component in percentage of the total score; and the calculations for the final score.

In most radiological scoring systems the lungs are evaluated for increased volume, bronchial wall thickening, bronchiectasis, mucus plugging, atelectasis and consolidation. In addition, for instance abscesses, bullae, septal thickening, mosaic perfusion, ground glass opacities and air trapping are evaluated in some CT scoring systems. Pleural affection and perfusion defects are scored on MRI.

Bronchiectasis alone, or in combination with mucus plugging, is given the highest weighting in most scoring systems and is thus commonly considered to be the most significant finding when evaluating cystic fibrosis lung disease.

Scoring of examinations is used for comparison of outcome in studies.

• chest x-ray
• quantitative radiography
• pulmonary function

• Air
• Bronchoalveolar Lavage/adverse effects
• Child
• Cystic Fibrosis/diagnosis
• Cystic Fibrosis/diagnostic imaging
• Female
• Humans
• Infant
• Infant, Newborn
• Lung Injury/etiology
• Lung Injury/pathology
• Male
• Neonatal Screening
• Radiography, Thoracic
• Respiratory Function Tests
• Retrospective Studies
• Tomography, X-Ray Computed
• Ventilation