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目的 分析原发性胆汁性肝硬化(PBC)和自身免疫性肝炎(AIH)重叠综合征(PBC/AIH重叠综合征)患者的临床表现、肝组织病理学特征。 方法 采用国际自身免疫性肝炎小组新修订的描述性诊断标准和计分系统,从68例PBC患者中筛选出9例PBC/AIH重叠综合征患者,分析比较PBC/AIH重叠综合征和单纯PHC患者的临床表现、肝组织病理学特征。 结果 68例PBC患者中,PBC/AIH重叠综合征9例,单纯PBC 59例,分别占13.2％和86.8％,PBC/AIH重叠综合征血清丙氨酸氨基转移酶、天冬氨酸氨基转移酶、γ-球蛋白、免疫球蛋白G,以及抗平滑肌抗体和(或)抗核抗体阳性率明显高于单纯PBC;PBC/AIH重叠综合征合并其他自身免疫性疾病发生率亦明显高于单纯PBC;PBC/AIH重叠综合征兼有PBC和AIH的双重组织学特点,显示界面炎和碎屑样坏死,肝组织免疫荧光检测显示肝细胞膜、汇管区有免疫球蛋白G沉着。 结论 PBC/AIH重叠综合征表现出A·IH和PBC双重特征,不同于单纯的PBC,两者的临床表现、免疫学及肝组织病理学特征上有差异。
Objective To analyze the clinical manifestations and histopathological features of patients with primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) overlap syndrome (PBC / AIH overlap syndrome). Methods Ninety-nine patients with PBC / AIH overlap syndrome were screened from 68 patients with PBC using the newly revised descriptive diagnostic criteria and scoring system of international autoimmune hepatitis group. The patients with PBC / AIH overlap syndrome and simple PHC were analyzed and compared The clinical manifestations, liver histopathological features. Results Sixty-eight patients with PBC had 9 cases of PBC / AIH overlap syndrome and 59 cases of simple PBC, accounting for 13.2% and 86.8% respectively. Serum alanine aminotransferase, aspartate aminotransferase , Γ-globulin, immunoglobulin G, and the anti-smooth muscle antibodies and (or) anti-nuclear antibody positive rate was significantly higher than PBC alone; PBC / AIH overlap syndrome with other autoimmune diseases were also significantly higher incidence of PBC ; PBC / AIH overlap syndrome both PBC and AIH double histological features, showing interface inflammation and debris-like necrosis, liver tissue immunofluorescence showed liver cell membrane, portal area immunoglobulin G calm. Conclusions The PBC / AIH overlap syndrome shows the dual characteristics of A · IH and PBC, which is different from the simple PBC. The clinical manifestations, immunology and pathological features of both PBC / AIH overlap syndromes are different.