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

关键词:  原发性胆汁性肝硬化  自身免疫性肝病  病理学

Key words:  primary biliary cirrhosis  anto-immum liver disease  pathology

摘要:

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阳性捡出率为55．4%（31/56）。其中AMA—M2阳性组和AMA—M2阴性组均以ALP、γ-GT、IgM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。

Abstract:

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 andⅡstage 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.