原发性胆汁性肝硬化93例临床及病理特点分析

目的总结原发性胆汁性肝硬化（PBC）患者的临床及病理学特点，以加深对该病的认识，指导临床诊疗。方法回顾性分析93例经肝活组织穿刺病理检查确诊为PBC患者的临床表现、生化指标、免疫学指标、病理学特点以及不同病理分期与生化指标相关性。结果93例患者临床症状中以乏力、皮肤瘙痒、黄疸最为常见。PBC患者均有不同程度肝功能异常，以ALP、GGT升高最为多见，异常比例分别为94．6％、95．7％。69．9％患者血清AMA和（或）AMA—M2亚型阳性，64．5％患者ANA阳性。93例肝脏病理学检查，符合I期为25例，Ⅱ期37例，Ⅲ期21例，Ⅳ期10例。病理分期与TBil、TBA呈正相关，与CHE呈负相关（P〈0．05）。结论诊断PBC应重视病理学检查，对病情及预后判断可参考TBil、TBA、CHE等指标，也应综合分析其临床症状、生化指标、自身抗体检测及组织学检查等。

Clinical and pathological characteristics in 93 cases with primary biliary cirrhosis

Objective To summarize the clinical and pathological characteristics in patients with primary biliary cirrhosis （PBC） , in order to get a better understanding of this disease and to guide the clinical diagnosis and treatment. Methods It was retrospectively analyzed that clinical manifestation, biochemical indexes, immunological and pathological characteristics, and correlation between pathological stage and biochemical indexes in 93 PBC patients diagnosed by liver biopsy. Results The most common symptoms of the 93 patients were fatigue, skin pruritus and jaundice. The biochemical indexes were abnormal in different degree. ALP and GGT were the most commonly abnormal indexes with the abnormal rates of 94.6% and 95.7% respectively. The positive rates of serum AMA and/or AMA-M2 were 69.9% ,while 64.5% of patients have the positive results of ANA. As to the liver pathology, 25 patients were at stage I, 37 patients at stage II, 21 patients at stage III, and 10 patients at stage IV. The pathological stages were positively correlated with TBil and TBA,and negatively correlated with CHE（P~0.05）. Conclusion We should pay attention to the examination of histopathology of PBC diagnosis. Judgement of the disease condition and prognosis can refer to the indexes of TBil, TBA, CHE, and compre hensively analyze the clinical symptoms, biochemical indexes, autoantibody detection and histological examination.