原发性硬化性胆管炎的临床和影像学特征分析  

Clinical and imaging features of primary sclerosing cholangitis

作　　者：于建安 贾翠宇[1] 武志岗 赵大伟[1] 冯骥良[1] 宋文艳[1] 李宏军[1] Yu Jian'an;Jia Cuiyu;Wu Zhigang;Zhao Dawei;Feng Jiliang;Song Wenyan;Li Hongjun(Department of Radiology,Beijing Youan Hospital,Capital Medical University,Beijing 100069,China)

机构地区：[1]首都医科大学附属北京佑安医院放射科,100069

出　　处：《北京医学》2022年第10期897-901,906,共6页Beijing Medical Journal

摘　　要：目的探讨原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)的临床及影像学特点,为PSC诊断和预后提供科学依据。方法选取2013年5月至2021年12月首都医科大学附属北京佑安医院诊断为PSC的40例患者,对其临床资料、实验室检查、影像学表现进行回顾性分析。结果40例患者男女比例为1.2∶1,平均年龄为52.4岁,临床症状以腹痛和腹胀、疲乏为主,合并炎性肠病5例,糖尿病7例。肝功能异常39例(97.5%),其中ALT升高21例,AST升高34例,T-BIL升高32例,GGT升高35例,碱性磷酸酶(alkaline phosphatase,ALP)升高34例,CA19-9升高21例。抗线粒体抗体(anti-mitochondrial antibody,AMA)阳性2例(5.0%),抗核抗体(antinuclear antibody,ANA)阳性25例(62.5%),二者均阳性3例(7.5%)。磁共振胰胆管造影(magnetic resonance cholangiopancreatography,MRCP)及内镜逆行胰胆管造影(endoscopic retrograde cholangiopancreatography,ERCP)显示,肝左叶、右叶肝内胆管均累及且呈串珠状表现36例(90.0%),单独肝右叶肝内胆管累及2例(5.0%),单独肝左叶肝内胆管累及1例(2.5%),肝内外胆管无扩张1例(2.5%);肝内外胆管均异常9例(22.5%)。CT增强及MRI表现以肝叶为基础的结节样增生15例(37.5%),包括弥漫性增大2例、尾状叶增大7例、尾状叶+右叶增大3例、尾状叶+左叶增大1例及单独左叶增大2例。其他影像学表现包括肝硬化28例(70.0%),肝内不均质强化14例(35.0%),胆囊壁增厚并明显强化15例(37.5%),胆囊切除8例(20.0%)等。结论PSC缺乏特异性的临床表现和实验室指标,当影像学检查出现肝内或肝内外胆管串珠状表现及肝叶异常比例增生时,应高度提示PSC的可能。Objective To explore the clinical and imaging features of primary sclerosing cholangitis(PSC),and provide scientific basis for the diagnosis and prognosis of PSC.Methods A total of 40 patients with PSC diagnosed in Beijing Youan Hospital,Capital Medical University from May 2013 to December 2021 were selected.The clinical data,laboratory examination,imaging results were retrospectively analyzed.Results The male to female ratio of the 40 patients was 1.2∶1,and the average age was 52.4 years old.The main clinical symptoms were abdominal pain,abdominal distension and fatigue.There were five cases of inflammatory bowel disease and seven cases of diabetes.Liver function was abnormal in 39 cases(97.5%),including increased ALT in 21 cases,increased AST in 34 cases,increased T-BIL in 32 cases,increased GGT in 35 cases,increased alkaline phosphatase(ALP)in 34 cases and increased CA19-9 in 21 cases.Two cases(5.0%)were positive for anti-mitochondrial antibody(AMA),25 cases(62.5%)were positive for antinuclear antibody(ANA),and three cases(7.5%)were positive for both AMA and ANA.Magnetic resonance cholangiopancreatography(MRCP)and endoscopic retrograde cholangiopancreatography(ERCP)showed that the intrahepatic bile ducts in the left and right lobe of the liver were involved in 36 cases(90.0%)with beaded performance,the intrahepatic bile ducts in the right/left lobe of the liver alone were involved in two cases(5.0%)/one case(2.5%),respectively.The intrahepatic and extrahepatic bile ducts were not dilated in one case(2.5%),and the intrahepatic and extrahepatic bile ducts were abnormal in nine cases(22.5%).Enhanced CT and MRI showed nodular hyperplasia based on hepatic lobe in 15 cases(37.5%),including diffuse enlargement in two cases,caudate lobe enlargement in seven cases,caudate lobe plus right lobe enlargement in three cases,caudate lobe plus left lobe enlargement in one case and left lobe enlargement alone in two cases.Other imaging findings included liver cirrhosis in 28 cases(70.0%),intrahepatic heterogeneity enhancemen

关 键 词：原发性硬化性胆管炎 自身免疫性肝炎 影像学特点 磁共振胰胆管造影 内镜逆行胰胆管造影 CT MRI 

分 类 号：R575.7[医药卫生—消化系统]