原发性硬化性胆管炎概念.ppt

原发性硬化性胆管炎 原发性硬化性胆管炎（primary sclerosing cholangitis）: 一种特发性於胆性疾病。胆管弥漫性炎症，广泛纤维化增厚和狭窄是本病的特征。 胆管病变可为均一性、节段性或不规则性。病变可累计整个胆道系统，以肝外胆管病变明显。 胆囊一般不受侵犯。 可逐渐发展致胆汁性肝硬化、门脉高压、肝衰竭而死亡。 病因 病因不明。目前认为与自身免疫性疾病、慢性肠源性感染、中毒等因素有关。约50%~70%病人合并有溃疡性结肠炎(BD)。 临床表现 本病少见，约2/3发生在45岁以下病人，男女之比约为3：2。 起病缓慢，黄疸初期呈间歇性加重，后期呈慢性进行性持续性梗阻，伴瘙痒及间歇性右上腹隐痛、恶心呕吐、乏力、体重减轻等，偶有畏寒发热等胆管炎表现。 常出现肝硬化、门脉高压症的表现。 病人常死于肝衰竭。 影像学表现 ERCP、MRCP显示胆管普遍性或局限性狭窄，或呈节段性多处狭窄，以肝管分叉部明显。病变累及肝内胆管时，肝内胆管分支减少并僵直，具有诊断价值。PTC常难以成功。 Cholangiographic classi?cation system for primary sclerosing cholangitis。 Intrahepatic Type 0 No abnormalities Type I Multiple strictures with normal caliber of the bile ducts or minimal dilatations Type II Multiple short, bandlike strictures, saccular dilatations, decreased arborisation Type III Despite adequate ?lling pressure only central branches ?lled; severe pruning, one or more outpouchings Extrahepatic Type 0 No abnormalities Type I Irregularities of extrahepatic duct contour, without distant narrowing Type II Segmental stenosis of extrahepatic duct, with smooth or irregular margin Type III Irregular stenosis and beading of almost entire length of the common duct Type IV Extremely irregular margin of the extrahepatic duct, diverticulumlike outpoutchings 影像学分型 肝内胆管分4型： T0型，肝内胆管未见异常。 I型，肝内胆管多发狭窄，狭窄间胆管正常或轻度扩张。 II型，肝内胆管多发狭窄，狭窄间胆管似小囊状扩张，呈串珠样改变。 III型，加压注入对比剂后肝内胆管仅中央主要分支充盈，远侧分支呈剪枝样。 肝外胆管分5型： 0型，肝外胆管未见异常。 I型，胆管无明显狭窄，仅边缘轻度不规则。 II型，胆管呈节段性狭窄，多累及肝总管或胆总管近端，范围2～4 cm。 III型，肝外胆管大部或全部受累，呈弥漫性狭窄。 IV型，胆管边缘很不规整，甚至呈憩室样凸出。 Secondary biliary sclerosis can mimick cholangiographic features of PSC. (A) The cholangiogram of a patient with ischemic-like cholangiopathy and biliary cast formation after prolonged anamnestic polytrauma with sepsis and mechanical ventilation. (B) A biliary cast that had been removed from the hepatic duct in this patient. ERCP with the corresponding MRCP of two patients with PSC. Patient A presents with multifocal strictures of the intrahepatic bile ducts and with a high-grade stenosis at the cy