Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation

Abstract

Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine. Chest X-ray (CXR), computed tomography (CT) and magnetic resonance imaging (MRI) are now available as routine modalities, each with individual strengths and drawbacks, which need to be considered when choosing the optimal modality adapted to the clinical situation of the patient. CT stands out with the highest morphological detail and has often been a substitute for CXR for regular severity monitoring at specialized centers. Multidetector CT data can be post-processed with dedicated software for a detailed measurement of airway dimensions and bronchiectasis and potentially a more objective and precise grading of disease severity. However, changing to CT was inseparably accompanied by an increase in radiation exposure of CF patients, a young population with high sensitivity to ionizing radiation and lifetime accumulation of dose. MRI as a cross-sectional imaging modality free of ionizing radiation can depict morphological hallmarks of CF lung disease at lower spatial resolution but excels with comprehensive functional lung imaging, with time-resolved perfusion imaging currently being most valuable.

Key Points:

• Hallmarks are bronchiectasis, mucus plugging, air trapping, perfusion abnormalities, and emphysema.

• Imaging is more sensitive to disease progression than lung function testing.

• CT provides the highest morphological detail but is associated with radiation exposure.

• MRI shows comparable sensitivity for morphology but excels with additional functional information.

• MRI sensitively depicts reversible abnormalities such as mucus plugging and perfusion abnormalities.

Citation Format:

• Wielpütz MO, Eichinger M, Biederer J et al. Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation. Fortschr Röntgenstr 2016; 188: 834 – 845

Zusammenfassung

Die progressive Lungenerkrankung bestimmt Morbidität und Mortalität der autosomal-rezessiv vererbten Mukoviszidose (Cystische Fibrose, CF). Die Implementierung der CF in das Neugeborenen-Screening erlaubt eine Diagnosestellung häufig bereits in einem präsymptomatischen Stadium. Verbesserungen der Therapie haben zudem eine stetig zunehmende Lebenserwartung ermöglicht, sodass die Mehrzahl der Patienten heute erwachsen ist. Da bildgebende Verfahren detaillierte Informationen über den regionalen Krankheitsverlauf bieten, werden heute Kontrollen in regelmäßigen Abständen empfohlen. Röntgenaufnahmen des Thorax, die Computertomografie (CT) und die Magnetresonanztomografie (MRT) stehen zur Verfügung – jedes Verfahren mit spezifischen Stärken und Schwächen, sodass die Wahl des Verfahrens an die individuelle klinische Situation des Patienten angepasst werden kann. Die CT bietet die höchste Detailauflösung und kann mittels Software nachverarbeitet werden, welche Atemwegsveränderungen quantitativ erfassen kann und potenziell eine objektivere Schweregradeinteilung ermöglicht. Die CT hat daher die Röntgenaufnahme an spezialisierten Zentren weitgehend abgelöst. Entsprechend ist die Strahlenexposition der CF-Erkrankten angestiegen, die altersbedingt besonders sensibel für ionisierende Strahlen sind und während ihres Lebens eine relevante Dosis akkumulieren können. Die MRT als alternatives strahlungsfreies Schnittbildverfahren stellt die typischen morphologischen Veränderungen der CF mit vergleichbarer klinischer Information bei etwas geringerer Detailauflösung dar. Mehr als jedes andere Verfahren ermöglicht die MRT eine Beurteilung der regionalen Lungenfunktion, wobei sich die zeitlich hoch aufgelöste Perfusions-MRT als praktikabel erwiesen hat.

Kernaussagen:

• Bildgebende Zeichen der Mukoviszidose sind Bronchiektasen, Mukoidimpaktionen, Air-Trapping, Perfusionsstörungen und Emphysem.

• Die Bildgebung ist sensitiver als die Lungenfunktionsprüfung für die Beurteilung der Krankheitsprogression.

• Die CT hat die höchste morphologische Auflösung, jedoch begleitet von bedeutsamer Strahlenexposition.

• Die MRT zeigt vergleichbare morphologische Details, ihre Stärke sind zusätzliche funktionelle Informationen.

• Die MRT stellt reversible Veränderungen wie Mukoidimpaktionen und Perfusionsstörungen sensitiv dar.

Key words

thorax - CT-quantitative - tracheobronchial tree - MR-functional imaging - fibrosis, cystic

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