Department of Diagnostic and Interventional Radiology, University Hospital of Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany.
Department of Paediatrics I, University Children's Hospital of Tuebingen, Tuebingen, Germany.
Eur Radiol. 2018 Jan;28(1):74-84. doi: 10.1007/s00330-017-4870-4. Epub 2017 Jun 29.
To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease.
This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function.
The inter-rater agreement of MR-CF-S was sufficient (mean intraclass correlation coefficient 0.92). MR-CF-S (-0.62; p < 0.05) and most of the categories significantly correlated with FEV. Differences between patients with relevant loss of FEV (>3%/year) and normal course were only significant for MR-CF-S (p < 0.05) but not for clinical parameters. Centrilobular opacity (CO) was the most promising score category for prediction of a decline of FEV (area under curve: whole cohort 0.69; subgroup 0.86).
MR-CF-S is promising to predict a loss of lung function. CO seems to be a particular finding in CF patients with an abnormal course.
• Lung imaging is essential in the diagnostic work-up of CF patients • MRI serves as a powerful, radiation-free modality in paediatric CF patients • Observational single-centre study showed significant correlation of MR-CF score and FEV • MR-CF score is promising in predicting a loss of lung function.
评估半定量 MRI 评分系统（MR-CF-S）作为囊性纤维化（CF）肺部疾病临床病程的预后标志物的适用性。
这项单中心 CF 队列的观察性研究纳入了一组 61 名患者（平均年龄 12.9±4.7 岁），他们接受了形态和功能肺部 MRI、肺功能测试（PFT）和 2 年的随访。MRI 由 3 位评分者使用 MR-CF-S 进行分析。评估了整个队列和 40 名肺功能中度受损患者亚组的评分者间一致性、评分类别与基线时 1 秒用力呼气量（FEV）的相关性，以及临床参数和评分类别的预测价值。
MR-CF-S 的评分者间一致性是足够的（平均组内相关系数 0.92）。MR-CF-S（-0.62；p<0.05）和大多数类别与 FEV 显著相关。只有 MR-CF-S （p<0.05）而不是临床参数在 FEV 相关丢失（>3%/年）和正常病程的患者之间存在差异。小叶中心性混浊（CO）是预测 FEV 下降的最有前途的评分类别（整个队列的曲线下面积：0.69；亚组 0.86）。
MR-CF-S 有望预测肺功能丧失。CO 似乎是 CF 患者中异常病程的一个特定表现。
•肺部影像学是 CF 患者诊断的重要组成部分。•MRI 是儿科 CF 患者的一种强大、无辐射的检查方法。•观察性单中心研究显示 MR-CF 评分与 FEV 显著相关。•MR-CF 评分有望预测肺功能丧失。
