硬化性胆管炎的影像诊断和鉴别诊断

摘要

硬化性胆管炎包括一系列以肝内外胆管炎症、纤维化和狭窄为特征的慢性及进展性胆汁郁积性肝病。硬化性胆管炎可以分为原发性硬化性胆管炎(PSC)和继发性硬化性胆管炎，前者病因未明；后者致病原因不同，包括IgG4-相关性硬化性胆管炎、复发性化脓性胆管炎、缺血性胆管炎、获得性免疫缺陷综合征相关性胆管炎和嗜酸性胆管炎。本研究在文献评阅的基础上，综合了不同系列硬化性胆管炎的影像表现，强调用系统方法鉴别诊断，提示支持诊断恶性胆道狭窄而非硬化性胆管炎的影像特征为：①门静脉期相对肝实质明显强化的狭窄节段；②长的狭窄长度(＞12 mm)；③明显的胆管壁增厚(＞3 mm)，不清楚的外边缘；④管腔不规则；以及不对称狭窄(70)。排除恶性胆道狭窄后，才是区分PSC和继发性硬化性胆管炎；还应结合人口统计、实验室检查和患者既往病史一并考虑。同时也讨论了磁共振胆管造影和内镜胆管造影在硬化性胆管炎诊断和鉴别诊断中作用以及治疗选项的临床意义。%Sclerosing cholangitis includes a spectrum of chronic, variably progressive cholestatic liver disease characterized by inflammation, fibrosis and stricture of the intrahepatic and extrahepatic bile ducts. Sclerosing cholangitis can be divided into primary sclerosing cholangitis of unidentified etiology and secondary sclerosing cholangitis caused by various identifiable etiologies, including immunoglobulin G4-related sclerosing cholangitis, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndromerelated cholangitis and eosinophilic cholangitis. In this article, we describe the imaging findings of the various spectrums of sclerosing cholangitis with anemphasis on a systemic approach in differential diagnosis. It is very important for magnetic resonance cholangiography and endoscopic cholangiography in imaging diagnosis and differential diagnosis of sclerosing cholangitis. We also discuss the clinical significance and therapeutic options for treating sclerosing cholangitis.