Epatocentro Ticino, Lugano, Switzerland.
Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK.
J Autoimmun. 2018 Dec;95:15-25. doi: 10.1016/j.jaut.2018.10.008. Epub 2018 Oct 23.
Juvenile sclerosing cholangitis is a rare chronic hepatobiliary disorder characterized by inflammation of the intra- and/or extrahepatic bile ducts, bile duct dilatation, narrowing and obliteration, and, histologically, by inflammatory bile duct damage leading to periductular fibrosis. The diagnosis is based on endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography. In children, it may be associated to a variety of systemic and hepatic conditions: thus, the term "primary" sclerosing cholangitis should be reserved for the rare cases without a known cause. Small duct disease is diagnosed in the presence of histological features diagnostic of sclerosing cholangitis and normal cholangiography. Autoimmune sclerosing cholangitis (ASC) is a form of sclerosing cholangitis with strong autoimmune features overlapping with those of autoimmune hepatitis (AIH). It is a well-recognized nosological entity in paediatrics, where it accounts for the majority of sclerosing cholangitis cases. It is as prevalent as AIH in children, is equally frequent in males and females, half of the patients have concomitant inflammatory bowel disease, virtually all patients have raised immunoglobulin G levels and positive anti-nuclear and/or anti-smooth muscle antibodies. Half of the ASC patients respond well to standard immunosuppressive treatment for AIH with the addition of ursodeoxycholic acid, but the transplant rate is higher than in AIH, and post-transplant recurrence is frequent. A number of open questions remain: are ASC and AIH distinct entities or different manifestations of the same condition? What is the role of histology? Is small duct disease a specific entity? What is the relationship between ASC and adult primary sclerosing cholangitis? What is the role of inflammatory bowel disease? In addition, validated diagnostic criteria for ASC are needed.
青少年原发性硬化性胆管炎是一种罕见的慢性肝胆疾病，其特征为肝内外胆管炎症、胆管扩张、狭窄和闭塞，组织学上表现为炎症性胆管损伤导致胆管周围纤维化。其诊断基于内镜逆行胰胆管造影或磁共振胰胆管造影。在儿童中，它可能与多种系统性和肝脏疾病有关：因此，“原发性”硬化性胆管炎的术语应保留给那些无已知病因的罕见病例。小胆管疾病的诊断依据为存在组织学特征诊断为硬化性胆管炎和正常胆管造影。自身免疫性硬化性胆管炎（ASC）是一种具有强烈自身免疫特征的硬化性胆管炎，与自身免疫性肝炎（AIH）重叠。在儿科中，它是一种公认的疾病实体，占硬化性胆管炎病例的大多数。它在儿童中的患病率与 AIH 相当，在男性和女性中同样常见，一半的患者伴有炎症性肠病，几乎所有患者的免疫球蛋白 G 水平升高，抗核抗体和/或抗平滑肌抗体阳性。半数 ASC 患者对 AIH 的标准免疫抑制治疗加用熊去氧胆酸反应良好，但移植率高于 AIH，且移植后复发频繁。仍有许多悬而未决的问题：ASC 和 AIH 是不同的实体还是同一疾病的不同表现？组织学的作用是什么？小胆管疾病是一种特定的实体吗？ASC 与成人原发性硬化性胆管炎的关系是什么？炎症性肠病的作用是什么？此外，还需要 ASC 的验证诊断标准。
