6O例原发性胆汁性肝硬化患者的临床与病理特征及预后分析

Liver histopathology, clinical features and prognostic factors of primary biliary cirrhosis： an analysis of 60 cases

目的了解原发性胆汁性肝硬化患者的临床与病理特征及影响生存的危险因素。方法分析60例原发性胆汁性肝硬化患者的一般资料、临床表现、生物化学、免疫学及病理学改变。患者全部定期随访，选择年龄、性别、白蛋白、胆红素、肝组织学分期5个可能对该病预后产生影响的研究因素，通过Cox比例风险模型对患者预后进行多因素分析。结果60例PBC患者中，女性与男性之比为4：1，平均年龄（52．5±9．4）岁。临床主要表现为黄疸（61．6％）、乏力（51．6％）、纳差（43．3％）、皮肤瘙痒（25％）。88．3％（53／60）的患者血清碱性磷酸酶及γ-谷氨酰转肽酶水平明显升高，分别为（242．3±137．1）U／L和（250．6±216．1）U／L，而血清ALT、AST水平仅轻或中度升高，分别为（185．8±269．1）U／L和（172．5±163．6）U／L，53．3％（32／60）的患者血清总胆红素≥34．2μmol／L，59．6％（28／47）的患者血清IgM升高，68．3％的患者（41／60）线粒体抗体和（或）线粒体抗体M2亚型阳性。有42例患者行肝脏病理学检查，早期（I、Ⅱ期）占33．3％，晚期（Ⅲ、Ⅳ期）占66．6％。随访：5例死亡，死亡原因为肝功能衰竭（3例）或上消化道出血（2例），45例生存，10例失访，平均生存期为3．92年。Cox多因素分析表明，影响预后的独立因素是年龄、胆红素、肝组织学分期。结论提高原发性胆汁性肝硬化的早期诊断，给予及时有效治疗，可改进预后和生存质量。

Objective Clinical and liver pathological features of 60 primary biliary cirrhrosis （PBC） patients were reviewed to identify prognostic factors in order to improve the diagnosis and treatment of the disease. Methods The general conditions, clinical manifestations, serum biochemical and immunological changes, and liver pathological findings were assessed in 60 PBC patients. All cases were followed up and 5 variables were studied by univariate analysis; the variables linked with survival were included in a Cox model. Results Forty-eight patients were females （80%）, 12 were males （20%）, and the mean age at their diagnoses was （52.5±9.4）. The symptoms most frequently complained about were jaundice （61.6%）, fatigue （51.6%）, anorexia （43.3%） and pruritus （25%）. Serum alkaline phosphatase （ALP） and glutamyl transpeptidase （GGT） levels were markedly elevated in the majority of the patients [（242.3±137.1） U/L and （250.6± 216.1 ） U/L, respectively], whereas ALT and AST levels were mildly to moderately elevated [（ 185.8±269.1） U/L and （172.5±163.6） U/L, respectively]. Thirty-two patients （53.3%） had a total bilirubin level of ≥34.2μmol/L. Twenty-eight patients （59.5%） had elevated serum IgM and 41 patients （68.3%） were anti-mitochondrial antibody AMA/AMA-M2 positive. Forty-two of the 60 patients had liver biopsies. The liver pathological changes： 33.3% of the cases were in I or Ⅱ stage and 66.6% in Ⅲ or Ⅳ stage. The follow up results： Five patients died of liver failure or massive upper gastrointestinal bleeding; 45 were still alive; the average survival peroid was 3.92 years; 10 patients were lost in the follow-up. With multivariate analysis （Cox model）, age, level of total bilirubin and the stage of the liver pathological changes were found to be independent factors finked to the survival of the patients. Conclusion PBC may not be a rare liver disease in China. The awareness to recognize PBC is important in making an early diagnosis and treatment.