Terracciano L M, Patzina R A, Lehmann F S, Tornillo L, Cathomas G, Mhawech P, Vecchione R, Bianchi L
Department of Pathology, University of Basel, Switzerland.
Am J Clin Pathol. 2000 Nov;114(5):705-11. doi: 10.1309/BHYM-HMY5-VM1P-7GEG.
We retrospectively studied 42 liver biopsy specimens from 39 patients who met serologic and histologic criteria of autoimmune liver diseases. We found 10 cases of overlap syndrome (OLS), 10 autoimmune cholangitis (AIC), 10 primary biliary cirrhosis (PBC), and 9 autoimmune hepatitis (AIH) type 1. The following results were obtained: (1) Granulomas and biliary duct lesions were more prominent in PBC and AIC than in OLS and AIH. (2) Bile duct loss was not observed in AIH cases. (3) Features of hepatocellular damage such as piecemeal necrosis, spotty lobular necrosis, and confluent necrosis, were much more prevalent in OLS and AIH than in PBC and AIC. (4) HLA-DR antigen expression by hepatocytes was more frequent in AIH and OLS, whereas the expression of the same antigen by the bile duct epithelium was more frequent in PBC and AIC. We conclude there is a morphologic spectrum in autoimmune liver diseases, in which PBC forms one end of the spectrum, AIH the other, OLS the middle but closer clinically and histologically to AIH than to PBC, and AIC, which seems to be an antimitochondrial antibody-negative subtype of PBC.
我们回顾性研究了39例符合自身免疫性肝病血清学和组织学标准的患者的42份肝活检标本。我们发现10例重叠综合征（OLS）、10例自身免疫性胆管炎（AIC）、10例原发性胆汁性肝硬化（PBC）和9例1型自身免疫性肝炎（AIH）。获得了以下结果：（1）肉芽肿和胆管病变在PBC和AIC中比在OLS和AIH中更突出。（2）AIH病例中未观察到胆管丢失。（3）肝细胞损伤特征如碎片状坏死、点状小叶坏死和融合性坏死在OLS和AIH中比在PBC和AIC中更常见。（4）肝细胞HLA-DR抗原表达在AIH和OLS中更频繁，而胆管上皮中相同抗原的表达在PBC和AIC中更频繁。我们得出结论，自身免疫性肝病存在形态学谱，其中PBC构成谱的一端，AIH构成另一端，OLS在中间，但在临床和组织学上更接近AIH而非PBC，而AIC似乎是PBC的抗线粒体抗体阴性亚型。
