原发性硬化性胆管炎的临床及影像诊断

Clinical and imaging diagnosis of primary sclerosing cholangitis

目的:探讨原发性硬化性胆管炎(PSC)的临床及影像诊断。方法:回顾性分析15例PSC患者的临床表现、实验室检查及各种影像学检查特征。结果:15例PSC患者中,男性19例,女性6例,平均年龄19岁。临床表现以进行性黄疸、皮肤瘙痒、乏力、纳差为主,病程0.5～19年。碱性磷酸酶(ALP)Md=280U/L,谷氨酰胺基转肽酶(GGT)Md=300U/L。超声检查主要表现为胆管壁增厚、回声增强,胆管内径变细。CT检查表现为不同程度的肝内外胆管扩张、胆管壁增厚、钙化。核磁共振胰胆管造影术(MRCP)检查表现为肝外胆管狭窄及肝内胆管周围部狭窄伴扩张。内窥镜逆行胰胆管造影术(ERCP)检查13例患者均为阳性,表现为肝内胆管串珠样或剪枝样改变和肝外胆管憩室样凸出。8例纤维结肠镜检查6例合并溃疡性结肠炎。结论:原发性硬化性胆管炎好发于中年男性,临床表现以梗阻性黄疸,血清ALP、GGT明显升高为其显著特点,往往伴有溃疡性结肠炎。超声及CT检查对本病诊断具有提示和辅助诊断作用,确诊以ERCP为主,MRCP可弥补ERCP检查的不足并作为病变随访的有效方法。

To discuss the clinical and imaging diagnosis of primary sclerosing cholangitis（PSC）. Methods： The dinical features, experimental and imaging examination results in 15 cases with PSC were analyzed retrospectively and discussed. Results： Of 15 patients, 9 were male and 6 female, and the mean age was 45 years, Clinical syrnptoms included progressive jaundice, skin itch, fatigue and anorexia. The lahoratory tests showed the evaluation of ALP（280U/L） and GGT（300U/L）. The wall bile duct thickening with strong echo and narrow bile duct in the ultrasonography were observed in all cases. CT films showed a different dilation of intra - hepatic and extra- hepatic bile ducts, bile duct wall thickening and caldfication. The stenosis in extra - hepatic bile duct and peripheral bile duct with dilation were found in MRCP. 13 cases were positive in ERCP with bead - like or branches - like changes in the intra - hepatic bile duct and diverticulum - like protrusion in extra - hepatic bile duct. Of 8 patients with PSC at colonoscopy, 6 ones were accompanied with ulcerative colitis. Conclusion： PSC occurs mainly in middle - aged males, and its clinical manifestation was obstructive jaundice with high level of ALP and GGT, usually accompanied with ulcerative colitis. Ultrasonic and CT have an irwlieative and assistant function to the diagnosis of PSC, the final diagnosis of PSC should be made by ERCP, and MRCP can make up the diagnosis by ERCP and was useful for the following - up of PSC.