Wielpütz M O, Eichinger M, Biederer J, Wege S, Stahl M, Sommerburg O, Mall M A, Kauczor H U, Puderbach M
Department of Diagnostic and Interventional Radiology, Subdivision of Pulmonary Imaging, University Hospital of Heidelberg, Heidelberg, Germany.
Department of Pulmonology and Respiratory Medicine, Cystic Fibrosis Center, Thoraxklinik at the University Hospital of Heidelberg, Heidelberg, Germany.
Rofo. 2016 Sep;188(9):834-45. doi: 10.1055/s-0042-104936. Epub 2016 Apr 13.
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.
• 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.
囊性纤维化（CF）中的进行性肺部疾病是这种常染色体隐性遗传病的生命限制因素。CF新生儿筛查的实施日益增多，甚至在症状前阶段也能实现诊断。治疗方面的改进已使生存率显著提高，现在大多数患者已成年。影像学可提供有关CF肺部疾病区域分布的详细信息，因此建议在临床常规中采用纵向影像学进行疾病监测。胸部X线（CXR）、计算机断层扫描（CT）和磁共振成像（MRI）目前都是常规检查手段，每种都有各自的优缺点，在选择适合患者临床情况的最佳检查手段时需要加以考虑。CT以最高的形态细节脱颖而出，在专业中心常被用作替代CXR进行常规严重程度监测。多排CT数据可用专用软件进行后处理，以详细测量气道尺寸和支气管扩张情况，并可能对疾病严重程度进行更客观、精确的分级。然而，改用CT不可避免地伴随着CF患者辐射暴露的增加，CF患者是对电离辐射敏感且会终生累积剂量的年轻人群。MRI作为一种无电离辐射的断层成像方式，能以较低的空间分辨率描绘CF肺部疾病的形态特征，但在全面的肺部功能成像方面表现出色，目前时间分辨灌注成像最具价值。
• 特征包括支气管扩张、黏液阻塞、气体潴留、灌注异常和肺气肿。
• 影像学对疾病进展比肺功能测试更敏感。
• CT提供最高的形态细节，但与辐射暴露相关。
• MRI对形态的敏感性相当，但在提供额外功能信息方面表现出色。
• MRI能敏感地描绘可逆性异常，如黏液阻塞和灌注异常。
• Wielpütz MO, Eichinger M, Biederer J等。囊性纤维化肺部疾病的影像学及临床解读。Fortschr Röntgenstr 2016; 188: 834 - 845。
