AMA-M2阴性原发性胆汁性肝硬化临床及病理学特点

Clinical and pathological characters of AMA-M2 negetive primary biliary cirrhosis

目的分析56例原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)的临床、生化及病理学特点,探讨免疫学指标和肝穿病理对临床诊断的意义。方法对56例临床诊断为原发性胆汁性肝硬化患者的一般资料、临床表现、生化指标、免疫学及病理学等进行分析。结果 56例单纯PBC中女性占80.4%,平均年龄(49.1±13.1)岁。临床主要表现为乏力(26/56,46.4%)、黄疸(20/56,35.7%)、右上腹不适(19/56,33.9%),皮肤瘙痒(9/56,16.1%)。其中9例无明显自觉症状,AMA-M2阳性检出率为55.4%(31/56)。其中AMA-M2阳性组和AMA-M2阴性组均以ALP、γ-GT、lgM升高为主,两组的生化及免疫学指标(除ALP、γ-GT、IgA外)比较无统计学意义;病理学表现为胆管周围炎和小胆管损伤,两组皆以Ⅰ、Ⅱ期改变为主(54.8%&52%)。结论 AMA-M2阴性PBC(AIC)可能是AMA-M2阳性PBC的另一表现形式,而不是另一种不同的疾病。GP210对AMA-M2阳性和AMA-M2阴性的PBC特异性均强。对于AMA-M2阴性PBC和AMA-M2阳性PBC,肝穿病理学检查具有重要临床价值。

Objective To review the clinical, biochemical and pathological characters of patients with primary biliary cirrhosis （PBC）. Methods Fifty-six cases with PBC were analyzed including the clinical features, biochemical results, immune detection and pathological examination. Results In all 56 cases of PBC,there were 45 females（80.4% ）, with an average age of（49.1±13.1） years. The clinical symptoms included fatigue （26/56,46.4%）, jaundice （ 20/56,35.7% ）, and uncomfortableness in the right upper abdominal （ 19/56,33.9% ）. All cases had no clinical signs. The positive rate of AMA-M2 detection was 55.4 % （31/56）. Serum, ALP, γ-GT, and IgM were increased in both AMA- M2 positive group and AMA-M2 negative patients. There was no significant difference between the positive group and negative group in biochemical and immune measurements （expect ALP, γ-GT and IgA） between two groups. The pathological examination showed perio-cholangitis and small bile ducts injury, Ⅰ stage and Ⅱ stage were most common in both two groups（54.8% and 52% ）. Conclusion AMA-M2 negative PBC（AIC） may be another form of AMA-M2 positive PBC, it is not diagnosed with another disease. GP210 detection has specificity to both AMA-M2 positive and AMA-M2 PBC. Liver biopsy and pathological examination are important procedures in diagnosis of AMA-M2 positive and negative PBC.