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原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征临床与病理分析

Clinical and pathological characterization of the overlap syndrome of autoirnmune hepatitis and primary biliary cirrhosis
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摘要 目的总结原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征患者的临床及病理组织学特点。方法对10例原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征患者的临床及病理资料进行回顾性分析。结果本组患者均有肝功能酶谱的不同程度升高,血清IgG和IgM升高,4例血清抗线粒体抗体(antimitochondrial antibody-2,AMA-2)阳性,其中3例抗核抗体(antinuelear antibod-y,ANA)阳性,1例核心蛋白gp210抗体(gp210)阳性。10例肝穿刺活检示均有小胆管损害,肝细胞炎症活动度从中度到重度,纤维化程度从S2到S4。结论自身免疫性肝病重叠综合征患者中女性多见,在临床及病理组织学上兼有原发性胆汁性肝硬化和自身免疫性肝炎的双重特点,临床工作中需结合临床、生物化学、免疫学及病理情况及时作出准确诊断。 Objective To explore clinical and histopathological characteristics of primary biliarysis-autoimmune hepatitis overlap syndrome. Methods Clinical data and pathological findings of 10 patients were reviewed. Results Serum glutamine transpeptidase, alkaline phosphatase levels, eirrhoalanine transaminase, aspartate transaminase, and serum IgG and IgM were elevated in all the patients. Four patients were positive for anti-mitoehondrial antibody-2. Three patients were positive for anti-nuclear anti-body and one patient was positive for gp210. Liver biopsies in these patients revealed: 10 patients had bile duct lesion, active hepatitis ranged from moderate to severe, and fibrosis ranged from S2 to S4. Conclusions PBC-AIH overlap syndrome is more common in middle-aged women. It has the clinical and histopathologieal characteristics of both PBC and AIH. Accurate clinical presentation, biochemical and immune and prompt diagnosis of overlap syndrome patients should be based on the indexes ,and hepatic pathological changes.
出处 《胃肠病学和肝病学杂志》 CAS 2007年第6期538-539,542,共3页 Chinese Journal of Gastroenterology and Hepatology
关键词 肝硬化 胆汁性 肝炎 自身免疫性 重叠综合征 Liver cirrhosis Biliary Hepatitis Autoimmune Overlap syndrome
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参考文献4

  • 1Heathcote EJ. Management of primary biliary cirrhosi. The American Association for the Study of Liver Diseases practice guidelines[ J ]. Hepatology, 2000, 31 ( 4 ) : 1005-1013.
  • 2Alvarez F, Berg PA, Bianchi FB, et al. InternationalAutoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis[ J]. J Hepatol, 1999,31 ( 5 ) :929-938.
  • 3Chazouilleres O, Wendum D, Serfaty L, :et al. Primary biliary cirrhosis2autoimmune hepatitis overlap syndrome: clinical features and response to therapy [ J ]. Hepatology, 1998,28 ( 2 ) :296-301.
  • 4Poupon R. Autoimmune overlapping syndromes [ J ]. Clin Liver Dis, 2003,7(4) :865-878.

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