目的 探讨早期原发性胆汁性肝硬化患者的临床特征,以期提高临床医师对该病的认识.方法回顾性分析2007 年4月-2012年6月我科收治的24例早期原发性胆汁性肝硬化患者的临床资料.结果 本组24例患者中7例无任何临床症状,有症状的17例患者中只有2例到感染专科就诊,其他患者因他科疾病就诊而发现肝功能异常,主要就诊于风湿免疫科、皮肤科、内分泌科及肿瘤科.肝脏生化指标中γ-谷氨酰转肽酶(γ- GGT)、碱性磷酸酶(ALP)、丙氨酸氨基转移酶、天门冬氨酸氨基转移酶超过参考值上限10倍患者的例数分别为11例、1例、1例、0例,5～倍者分别为8例、5例、6例、5例,2～倍者分别为4例、16例、11例、12例,＜2倍者分别为1例、2例、6例、7例.相关免疫学指标中血清IgM水平升高19例,IgG升高5例,而血清IgA水平均正常;抗线粒体抗体(AMA)、抗线粒体抗体M2亚型(AMA-M2)均阳性.结论 早期原发性胆汁性肝硬化缺乏典型临床表现,多数患者因合并其他自身免疫性疾病而就诊,γ- GGT、IgM水平升高,AMA、AMA-M2阳性是其主要表现和诊断依据.%Objective To study the clinical features of early primary biliary cirrhosis, to improve its cognition and early diagnosis. Methods The clinical data of 24 patients with early primary biliary cirrhosis from April 2007 to June 2012, were analyzed retrospectively. Results Seven patients had no clinical symptoms, only 2 of the 17 symptomatic patients visited infection specialists, the others were diagnosed as abnormal liver function when visiting other departments, commonly departments of rheumatology, skin, endocrinology and oncology. In liver biochemical indexes, the numbers of patients who had over 10 times higher levels of gamma - glutamyl transpeptidase ( γ - GGT ), alkaline phosphatase ( ALP ), alanine aminotransferase, aspartate amino transferase than the upper reference limit were 11, 1, 1 , 0, respectively; Those who had over 5 times were 8, 5, 6, 5, respectively; Those who had over 2 times were 4, 16, 11, 12, respectively; Those who had 2 or fewer times higher were 1, 2, 6, 7, respectively. In related immunological parameters, 19 patients had higher serum IgM, 5 had elevated IgG, but they all had normal IgA. Antimitochondrial antibodies ( AMA ), AMA - M2 were positive. Conclusion Early primary biliary cirrhosis is lack of typical clinical features. Most of the patients visit doctors due to their cofigurative autoimmune diseases. Elevated serum γ - GGT, IgM, positive AMA/AMA - M2 are the main manifestations and its diagnostic base.
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