原发性胆汁性肝硬化自身免疫性肝炎重叠综合征临床病例分析

Clinical and pathological characteristics of primary biliary cirrhosis(PBC) and autoimmune hepatitis(AIH) overlap syndrome:an analysis of 11 cases

目的探讨原发性胆汁性肝硬化自身免疫性肝炎(PBCAIH)重叠综合征的临床病理特征及治疗应答。方法对具有肝穿刺标本的11例PBCAIH重叠综合征、21例Ⅰ型AIH和20例PBC(Scheuer分期Ⅰ、Ⅱ期)患者进行比较,重点分析PBCAIH重叠综合征的临床表现、病理特点及治疗应答。结果3组患者的性别、年龄、病程无显著差异;PBCAIH重叠综合征血清碱性磷酸酶(ALP)、γ谷氨酰转肽酶(GGT)、免疫球蛋白IgM以及抗线粒体抗体(AMA)、AMAM2阳性率明显高于AIH组(P<0.05或P<0.01);而丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)、γ球蛋白、免疫球蛋白IgG以及抗核抗体(ANA)或抗平滑肌抗体(SMA)阳性率明显高于PBC(P<0.05或P<0.01)。肝组织学示界面炎/碎屑样坏死、小叶内炎症及胆管病变;重叠综合征熊去氧胆酸(UDCA治疗组7例中4例(57%)肝功能改善(P<0.05),UDCA及泼尼松龙联合治疗组7例中5例(71%)ALT、胆红素(BIL)、AST恢复正常,ALP、GGT明显下降(P<0.05或P<0.01)。PBC组中13例(65%)ALT、BIL、ALP、GGT明显下降(P<0.05)。结论PBCAI重叠综合征临床、血清学及组织病理学表现出AIH和PBC双重特征,不同于单纯的PBC和AIH,UDCA及泼尼松龙联合治疗有助于生化指标改善。

Objective To analyze the clinical and pathological features and responses to therapy of primary biliary cirrhosis（PBC） and autoimmune hepatitis（AIH） overlap syndrome. Methods Comparison was made between 11 patients with PBC/AIH overlap syndrome, 21 cases with type 1 AIH and 20 cases with PBC （Scheuer stage Ⅰ and Ⅱ）, and the emphases was laid upon the clinical manifestations, pathological features and responses to therapy of the patients with PBC/AIH overlap syndrome. Results No significant differences were found in sex, age and course of diseases among the three groups. In PBC/AIH group, the serum levels of alkaline phosphatase （AKP）, γ-glutamyl-transpeptidase （GTP）, IgM and the frequency of positive anti-mitochondrial antibodies （AMA） and AMA-M2 antibodies were significantly higher than those in the pure AIH group（P〈0. 05 or P〈0. 01）; and the levels of alanine transaminase （ALT）, aspartic transaminase （AST）, γ-globulin and IgG as well as the presence of positive antinuclear antibody （ANA） or anti-smooth muscle antibody （SMA） were markedly higher than those in PBC group （P〈0. 05 or P〈0. 01）. The liver biopsy specimens in the PBC/AIH group were characterized by interface hepatitis, piecemeal necrosis and bile duct lesion. At the end of follow-up survey, of 7 overlap syndrome patients treated by UDCA, liver function was improved significantly in 4 cases （P〈0. 05）. 5 of 7 patients who were given UDCA plus prednisolone had decreased ALT, AST, BIL, AKP and GTP （P〈0. 05 or P〈0. 01）. In PBC group, there were 13 （65%） patients whose ALT, AST, BIL, AKP, GTP decreased significantly （P〈0. 05）. Conclusion A combined features of both PBC and AIH existed in overlap syndrome, differed from PBC or AIH alone. The combined administration of UDCA plus prednisolone may benefit those patients with PBC/AIH overlap syndrome.