原发性胆汁性肝硬化的临床及病理学特征

Clinical and pathological characteristics of primary biliary cirrhosis

目的分析原发性胆汁性肝硬化患者的临床及病理学特征,以有助于提高对该病的认识及诊断. 方法对37例原发性胆汁性肝硬化患者临床资料及其中20例肝穿刺病理资料进行回顾性分析. 结果原发性胆汁性肝硬化患者中女性35例,确诊时平均年龄为 50.4±8.9岁,常见临床表现多为黄疸(70.3%)、乏力(70.3%)、瘙痒(56.8%).所有患者γ-谷氨酰转肽酶(GGT)与碱性磷酸酶均有显著升高(中位值分别为467.50U/L和424.00U/L).94.6%(35/37)患者血清总胆汁酸水平、86.5% (32/37) 患者血清胆固醇、86.5%(32/37)患者血清IgM有明显升高.91.9%(34/37)患者血清线粒体抗体和(或)线粒体抗体M2亚型阳性.20例原发性胆汁性肝硬化患者肝穿刺病理主要表现为:纤维化85%(17/20)、小叶间胆管损伤65%(13/20)、单核细胞炎症65%(13/20)、碎屑样坏死50%(10/20)、胆色素聚集45%(9/20).应用非参数检验分析示GGT升高与病理分期(Z＝-3.099,P＝0.002)及小叶间胆管损伤程度相关(Z＝2.655,P＝0.01). 结论原发性胆汁性肝硬化中年女性多见,临床工作中需综合临床、生物化学、免疫学及病理情况及时准确诊断.而GGT可部分反映PBC的组织学改变的严重程度.

Objective In order to improve the cognition and early diagnosis of primary biliary cirrhosis (PBC), we investigated clinical and pathological characteristics of PBC. Methods Clinical data of 37 PBC patients together with pathological findings of 20 PBC patients were reviewed. Results Among the 37 patients, 35 were women and the mean age at diagnosis was (50.4 ± 8.9) years. The most frequent clinical presentations were.jaundice (70.3%), fatigue (70.3%), and pruritus (56.8%). Serum glutamyl transpeptidase (GGT) and alkaline phosphatase (ALP) levels were markedly elevated in all patients (P50 was 467.50 U/L and 424.00 U/L, respectively). Among the 37 cases, 35 (94.6%) had total bile acids elevation, 32 (86.5%) had serum cholesterol elevation and 32 (86.5%) serum IgM elevation, 34 (91.9%) were positive for anti-mitochondrial antibody (AMA) and/or AMA-M2. Liver biopsy in 20 PBC patients mainly found: fibrosis in 17 cases (85%), in-terlobular bile duct lesions in 13 (65%), lobular mononuclear inflammation in 13 (65%), lymphocytic hepatocellular piecemeal necrosis in 10 (50%), and bile pigment accumulation in 9 (45%). The results of nonparametric test showed that GGT was related with pathological stage (Z=3.099, P=0.002) and interlobular bile duct lesions (Z=2.655, P=0.01). Conclusions PBC is mostly found in middle-aged women. Accurate and prompt diagnosis of PBC should be based on the clinical presentation, biochemical and immunological indexes, and hepatic pathological changes. The level of GGT may partly reflect the severity of the histological lesions.