目的：比较研究PBC/AIH重叠综合征和单纯PBC或AIH患者的临床表现、生化检查、免疫学检查及病理学特点。方法：选取自身免疫性肝病患者86例，对其一般资料、临床表现、实验室检查、病理学改变及治疗转归进行回顾性分析。结果：86例自身免疫性肝病患者中有10例AIH/PBC诊断为重叠综合征；生化检查其血清碱性磷酸酶、γ-谷胺酰氨基转移酶、总胆红素、直接胆红素、谷丙转氨酶及谷草转氨酶呈明显升高；特异性免疫指标ANA阳性6例，AMA阳性8例，AMA-M2阳性8例，SMA阳性2例；病理学特征表现有80%的患者可见界面炎/碎屑坏死，有患者出现桥接样坏死，纤维组织增生多见，70%的患者有浆细胞浸润，患者均有不同程度的胆管改变。结论：PBC/AIH重叠综合征不仅具备AIH的特点，同时还具备PBC的特点。%Objective:To study the features of clinic,biochemistry,immunology and pathology in PBC/AIH and PBC(or AIH). Methods:Eighty-six patients with autoimmunic liver disease were studied to compare PBC, AIH and PBC/AIH overlap syndome in the clinical symptoms, signs, labarotary findings and histological features. Results: Ten cases were diagnosed as AIH/PBC overlap syndrome in eighty-six patients with autoimmune liver disease.The biochemical tests results were the following indexes increased significantly in serum alkaline phosphatase, gamma glutamyl aminotransferase, total bilirubin, direct bilirubin, alanine aminotransferase as aspartate aminotransferase; The specific immunological marker were:ANA positive in six cases, AMA positive six cases, AMA-M2 positive in four cases, SMA positive two cases; The pathological features were found as: visible interface inflammation in 80% cases, clastic necrosis and bridging necrosis, fibrous tissue proliferation in some cases, plasma cell infiltration in 70% cases. In addition, all patients were observed the lesion of bile duct in different degree. Conclusion: PBC/AIH overlap syndrome has not only the characteristics of AIH, but also the characteristics of PBC.
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