原发性硬化性胆管炎(Primary Sclerosing Cholangitis.简称PSC)是一病因不明的少见疾病。临床上以肝内、外胆管系统弥漫性或节段性、非特异性炎症导致纤维增厚造成慢性梗阻性黄疸为特征。不易诊断,确诊主要靠手术和病理。诊断本病文献中亦称为“狭窄性胆管炎”闭塞性胆管炎”“硬化性胆管炎”“纤维性胆管炎”“总胆管非肿瘤性狭窄”等。多数作者同意称为“原发性硬化性胆管炎首例由Delbet(1924)报告。后陆续出现一 Primary Sclerosing Cholangitis (PSC) is a rare disease of unknown etiology. Clinically, it is characterized by chronic obstructive jaundice caused by diffuse or segmental and non-specific inflammation of the intrahepatic and extrahepatic bile ducts, resulting in thickened fibers. Difficult to diagnose, confirmed mainly by surgery and pathology. Diagnosis of this disease literature is also known as “stenotic cholangitis” occlusive cholangitis, “sclerosing cholangitis,” “fibrillary cholangitis,” “non-neoplastic stenosis of the common bile duct,” etc. Most authors agree to call it "primary The first case of sclerosing cholangitis was reported by Delbet (1924). One after another