Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Abdom Radiol (NY). 2020 Jan;45(1):168-176. doi: 10.1007/s00261-019-02184-z.
Autoimmune liver diseases (AILD), including primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH), have overlapping clinical features but distinct management strategies and outcomes. The purpose of this study was to assess the diagnostic performance of quantitative magnetic resonance cholangiopancreatography (MRCP) parameters for distinguishing PSC/ASC from AIH in children and young adults.
This IRB-approved, cross-sectional study included participants from an institutional AILD registry that underwent baseline serum liver biochemistry testing and 3D fast spin-echo MRCP. The biliary tree was extracted and modeled from MRCP images using novel proprietary software (MRCP+ ™; Perspectum Diagnostics; Oxford, United Kingdom), and quantitative parameters were generated (e.g., biliary tree volume; number and length of bile ducts, strictures, and dilations; bile duct median/maximum diameters). Mann-Whitney U tests were performed to compare laboratory values and MRCP metrics between patient cohorts (clinical diagnosis of PSC/ASC versus AIH). Receiver operating characteristic (ROC) curves and multivariable logistic regression were used to assess diagnostic performance of serum biochemistry values and MRCP parameters for discriminating PSC/ASC from AIH.
Thirty percent (14/47) of MRCP exams failed post-processing due to motion artifact. The remaining 33 patients included 20 males and 13 females, with a mean age of 15.1 ± 3.9 years. Eighteen patients were assigned the clinical diagnosis of PSC or ASC and 15 of AIH. All but one quantitative MRCP parameter were significantly different between cohorts (p < 0.05) and predictive of diagnosis (ROC p < 0.05), including numbers of bile duct strictures (area under curve [AUC] = 0.86, p < 0.0001) and dilations (AUC = 0.87, p < 0.0001) and total length of dilated ducts (AUC = 0.89, p < 0.0001). Laboratory values were not significantly different between cohorts (p > 0.05). The best multivariable model for distinguishing PSC/ASC from AIH included total length of dilated ducts (odds ratio [OR], 1.08; 95% CI 1.02-1.14) and maximum left hepatic duct diameter (OR, 1.21; 95% CI 0.57-2.56) [AUC = 0.92].
Quantitative MRCP parameters provide good discrimination of PSC/ASC from AIH.
自身免疫性肝病（AILD），包括原发性硬化性胆管炎（PSC）、自身免疫性硬化性胆管炎（ASC）和自身免疫性肝炎（AIH），具有重叠的临床特征，但管理策略和结果不同。本研究旨在评估定量磁共振胰胆管造影（MRCP）参数在儿童和青年中鉴别 PSC/ASC 与 AIH 的诊断性能。
这项经过机构审查委员会批准的横断面研究纳入了来自一个自身免疫性肝病登记处的参与者，他们接受了基线血清肝功能检测和 3D 快速自旋回波 MRCP 检查。使用新的专有的软件（MRCP+TM；Perspectum Diagnostics；英国牛津）从 MRCP 图像中提取和建模胆管树，并生成定量参数（例如，胆管树体积；胆管数量和长度、狭窄和扩张；胆管的中值/最大直径）。采用 Mann-Whitney U 检验比较两组患者的实验室值和 MRCP 指标（PSC/ASC 的临床诊断与 AIH）。采用受试者工作特征（ROC）曲线和多变量逻辑回归评估血清生化值和 MRCP 参数对鉴别 PSC/ASC 与 AIH 的诊断性能。
30%（14/47）的 MRCP 检查因运动伪影而无法进行后处理。其余 33 例患者中包括 20 名男性和 13 名女性，平均年龄为 15.1±3.9 岁。18 例患者被诊断为 PSC 或 ASC，15 例患者被诊断为 AIH。除了一个定量 MRCP 参数外，所有参数在两组之间均有显著差异（p<0.05），并具有预测诊断的能力（ROC p<0.05），包括胆管狭窄数量（曲线下面积[AUC]为 0.86，p<0.0001）和扩张数量（AUC 为 0.87，p<0.0001）以及扩张胆管总长度（AUC 为 0.89，p<0.0001）。两组之间的实验室值无显著差异（p>0.05）。用于鉴别 PSC/ASC 与 AIH 的最佳多变量模型包括扩张胆管总长度（比值比[OR]，1.08；95%置信区间[CI]，1.02-1.14）和左肝内胆管最大直径（OR，1.21；95%CI，0.57-2.56）[AUC=0.92]。
定量 MRCP 参数可很好地区分 PSC/ASC 与 AIH。
