Washington Mary K
Department of Pathology, Vanderbilt University Medical Center, Nashville, TN, USA.
Mod Pathol. 2007 Feb;20 Suppl 1:S15-30. doi: 10.1038/modpathol.3800684.
The three main categories of autoimmune liver disease are autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC); all are well-defined entities with diagnosis based upon a constellation of clinical, serologic, and liver pathology findings. Although these diseases are considered autoimmune in nature, the etiology and possible environmental triggers of each remain obscure. The characteristic morphologic patterns of injury are a chronic hepatitis pattern of injury with prominent plasma cells in AIH, destruction of small intrahepatic bile ducts and canals of Hering in PBC, and periductal fibrosis and inflammation involving larger bile ducts with variable small duct damage in PSC. Serological findings include the presence of antimitochondrial antibodies in PBC, antinuclear, anti-smooth muscle, and anti-LKM antibodies in AIH, and pANCA in PSC. Although most cases of autoimmune liver disease fit readily into one of these three categories, overlap syndromes (primarily of AIH with PBC or PSC) may comprise up to 10% of cases, and variant syndromes such as antimitochondrial antibody-negative PBC also occur. Sequential syndromes with transition from one form of autoimmune liver disease to another are rare.
自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）；所有这些都是明确的疾病实体，其诊断基于一系列临床、血清学和肝脏病理学检查结果。尽管这些疾病本质上被认为是自身免疫性的，但每种疾病的病因和可能的环境触发因素仍不清楚。特征性的损伤形态模式为：AIH表现为伴有显著浆细胞的慢性肝炎损伤模式，PBC表现为肝内小胆管和赫林管的破坏，PSC表现为较大胆管的导管周围纤维化和炎症，伴有不同程度小胆管损伤。血清学检查结果包括：PBC中存在抗线粒体抗体，AIH中存在抗核抗体、抗平滑肌抗体和抗肝肾微粒体抗体，PSC中存在核周型抗中性粒细胞胞浆抗体（pANCA）。虽然大多数自身免疫性肝病病例很容易归入这三类中的一类，但重叠综合征（主要是AIH与PBC或PSC重叠）可能占病例的10%，也会出现如抗线粒体抗体阴性的PBC等变异综合征。从一种自身免疫性肝病形式转变为另一种形式的连续综合征很少见。
