Dienes H P
Institut für Pathologie der Universität zu Köln, 50924 Köln.
Verh Dtsch Ges Pathol. 2005;89:155-62.
Autoimmune liver diseases encompass autoimmune hepatitis, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) as lesions of the biliary tract. The term autoimmune cholangitis has not been generally accepted, so it remains an entitiy waiting for precise definition. AIH is a chronic progressive necroinflammatory liver disease mostly occuring in female individuals and leading to ultimate autodestruction of the liver if not treated. Histopathology of the liver reflects the gerneral understanding of the underlying immune especially self reactive CD4 + T-helper cells mediated mechanisms in destruction of liver cells displaying a typical but by no means pathognomonic histopathological pattern. Since there are no specific and generally valid tests the diagnosis should be confirmed by a scoring system including histopathology. Variants of autoimmune hepatitis cover seronegative cases, acute onset autoimmune hepatitis and autoimmune hepatitis with centrilobular necrosis. Differential diagnosis of autoimmune hepatitis includes drug induced chronic hepatitis that may mimick autoimmune hepatitis by clinical course and serology. Histopathology may give helpful hints for the correct diagnosis. Autoimmune lesions of the biliary tract are PBC in the first line. The target antigen of the autoimmune response has been identified, natural history of the diseases is well known and histopathology is pathognomonic in about a third of the cases. In clinical practice liver biopsy is taken to exclude other etiologies when AMA is present in the serum, staging the disease at first diagnosis and to establish diagnosis in cases of AMA negativity. The autoimmune nature of PSC has been discussed in the literature ever since the first description and the answer in not settled yet. Histopathology is relevant for the diagnosis in excluding other etiologies and confirming the diagnosis of small duct PSC. The term autoimmune cholangitis has been used to designate AMA-negative PBC, however, based on research experience and the clinical data it should be reserved to the overlap syndrome of AIH and PSC in children that seem to make up a disease entitiy of its own.
自身免疫性肝病包括自身免疫性肝炎、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）等胆道病变。自身免疫性胆管炎这一术语尚未被普遍接受，因此它仍是一个有待精确界定的实体。自身免疫性肝炎是一种慢性进行性坏死性炎症性肝病，主要发生于女性，若不治疗最终会导致肝脏自我破坏。肝脏的组织病理学反映了对潜在免疫机制的一般认识，尤其是自身反应性CD4 +辅助性T细胞介导的肝细胞破坏机制，呈现出一种典型但并非特异性的组织病理学模式。由于没有特异性且普遍有效的检测方法，诊断应通过包括组织病理学在内的评分系统来确认。自身免疫性肝炎的变体包括血清学阴性病例、急性起病的自身免疫性肝炎和伴有小叶中心坏死的自身免疫性肝炎。自身免疫性肝炎的鉴别诊断包括药物性慢性肝炎，其在临床病程和血清学方面可能会模仿自身免疫性肝炎。组织病理学可能为正确诊断提供有用线索。胆道的自身免疫性病变首先是PBC。自身免疫反应的靶抗原已被确定，该疾病的自然史已为人熟知，约三分之一病例的组织病理学具有特异性。在临床实践中，当血清中存在抗线粒体抗体（AMA）时，进行肝活检以排除其他病因，在初次诊断时对疾病进行分期，并在AMA阴性的病例中确立诊断。自首次描述以来，PSC的自身免疫性质在文献中一直存在讨论，目前尚无定论。组织病理学对于排除其他病因并确诊小胆管PSC具有重要意义。自身免疫性胆管炎这一术语曾被用于指代AMA阴性的PBC，然而，基于研究经验和临床数据，它应保留用于儿童中自身免疫性肝炎和PSC的重叠综合征，这似乎构成了一种独立的疾病实体。
