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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49例分析

杨蜜蜜1,王邦茂2,周璐1,张洁2,林睿1   

  1. 1. 天津医科大学总医院消化科
    2. 天津医科大学总医院
  • 收稿日期:2013-01-09 修回日期:2013-04-10 出版日期:2013-05-15 发布日期:2013-05-15
  • 通讯作者: 杨蜜蜜

Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome in 49 Cases Analysis

  • Received:2013-01-09 Revised:2013-04-10 Published:2013-05-15 Online:2013-05-15

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摘要：

【摘要】 目的 分析原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征(PBC-AIHOS)患者的临床特点及合并肝外自身免疫性疾病情况。方法 收集PBC-AIHOS患者49例,分析其血清学指标、肝组织病理学和影像学特点、肝硬化及合并肝外自身免疫性疾病情况。结果 49例PBC-AIHOS患者以女性(83.7%)为主,平均发病年龄(57.2±8.9)岁,均有不同程度生化指标升高:ALT(225.0±248.0)U/L、AST(207.6±233.1)U/L、GGT(409.6±326.2)U/L、ALP(338.8±274.8)U/L、TBIL(51.1±67.7)μmol/L;31例GLO[(42.9±10.6)g/L]升高。接受IgA、IgG、IgM水平检测的31例患者中,18例IgM[(3872±2945)mg/L]、19例IgG[(18426±6726)mg/L]水平升高;ANA、AMA、SMA阳性率分别为98.0%、89.8%、6.1%;初诊时肝硬化38.8%,其中失代偿期57.9%;合并肝外自身免疫性疾病40.8%;影像学检查示腹腔淋巴结肿大者91.8%。结论  PBC-AIHOS病理特点更倾向AIH,且易并存肝外自身免疫性疾病,常规评估应注意对其拓展筛查;腹腔淋巴结肿大可能对诊断PBC-AIHOS有指导意义。 

关键词: 胆管炎, 硬化性, 肝炎, 自身免疫性, 肝硬化, 胆汁性, 重叠综合征, 病理学

Abstract: Objection  To summary and analyze the clinic characteristics features and associated extrahepatic autoimmune diseases of patients with primary biliary cirrhosis -autoimmune hepatitis overlap syndrome(AIH-PBC OS). Methods   To analyze retrospectively the clinic characteristics and pathologic features of 49 patients that were diagnosed as PBC-AIH OS and been followed-up when been treated in digestive department of general hospital during Jan.2000 to Jan.2012. Results   These patients were most female with age of 51-60 years old, and the mean age of onset was 57.2±8.9 years old. The levels of serum ALT, AST, GGT and TBIL were increased in various degree in all the patients, while serum GLO/IgG was inceased in 79.6% patients. Positive rate of ANA,AMA and SMA was 98.0%,89.8% and 6.1% respectively. When been diagnosed, 38.8% of the patients had been developed to hepatic cirrhosis, 57.9%(11/19)of them at the stage of decompensation . Besides, pathologic stage was 3,4 in 16 (66.7%)patients with PBC and 1,2 stage in 8(33.3%)ones. Among them, there were 20 (40.8%)patients had extrahepatic autoimmune diseases at the same time, 16 cases (32.7%) had two kinds of autoimmune disease, 4 cases (8.1%) with three kinds of autoimmune diseases. Abdominal ultrasonic wave showed abnormality in 93.9% of patients,the abdominal lymph nodes were enlarged in 91.8% of the patients. Conclusion  Most the patients with PBC-AIH OS had been diagnosed as hepatic cirrhosis at the first time, so as to make early diagnosis and treatment. The symptoms were various and complicated so that it’s significant to expand the screening of incorporated autoimmune diseases. Abdominal lymph nodes enlargerment found by imaging examination may had guiding sense to diagnosis.

Key words: hepatitis, autoimmune diseases, hepatic cirrhosis, cholestasis, Overlap Syndrome, 病理学