Sherlock S
Lancet. 1987 Aug 29;2(8557):493-6. doi: 10.1016/s0140-6736(87)91802-2.
Diseases with disappearing intrahepatic bile ducts may be developmental, immunological, infective, vascular, or chemical in origin. The immunological group includes primary biliary cirrhosis, graft-versus-host disease, and sarcoidosis. HLA class 2 antigens are displayed on the bileducts and recognition of biliary antigens by cytotoxic T-cells leads to destruction of interlobular ducts. Primary sclerosing cholangitis is associated with immunological features, but the hepatic histology is not that of immunological duct disease. The association with immunodeficiency syndromes, and the finding that secondary sclerosing cholangitis may occur in patients with the acquired immunodeficiency syndrome who are infected with cytomegalovirus, suggest that primary sclerosing cholangitis might be infective in origin. In bacterial cholangitis there is contiguity between the biliary system and the intestinal tract and usually, but not necessarily, partial biliary obstruction. Interference with the hepatic arterial supply to the bileducts leads to vascular cholangitis. Chemical cholangitis follows injection of scolicidal agents into the biliary tree. Diseases with disappearing bileducts have a long natural history and hepatocellular failure occurs late. In the late stages hepatic transplantation gives good results.
肝内胆管消失性疾病的病因可能是发育性、免疫性、感染性、血管性或化学性的。免疫性疾病组包括原发性胆汁性肝硬化、移植物抗宿主病和结节病。HLA - Ⅱ类抗原在胆管上表达，细胞毒性T细胞对胆管抗原的识别导致小叶间胆管的破坏。原发性硬化性胆管炎与免疫特征相关，但肝脏组织学表现并非免疫性胆管疾病。与免疫缺陷综合征的关联，以及在感染巨细胞病毒的获得性免疫缺陷综合征患者中可能发生继发性硬化性胆管炎的发现，提示原发性硬化性胆管炎可能起源于感染。在细菌性胆管炎中，胆道系统与肠道相邻，通常但不一定存在部分胆道梗阻。肝动脉对胆管供血的干扰会导致血管性胆管炎。化学性胆管炎是在向胆管树注射杀绦虫剂后发生的。胆管消失性疾病有很长的自然病程，肝细胞衰竭出现较晚。在晚期，肝移植效果良好。
