35例自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征的临床特征

[Clinical features of 35 cases of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome].

作者信息

Zeng Zhen, Han Yu-Kun, Zhang Xiao-Feng, Geng Hua

机构信息

302 Hospital of PLA, Beijing 100039, China.

出版信息

Zhonghua Gan Zang Bing Za Zhi. 2005 Jan;13(1):3-5.

PMID:15670480

Abstract

OBJECTIVE

To realize the clinical features of autoimmune hepatitis-primary biliary cirrhosis (AIH+PBC) overlap syndrome.

METHODS

We analyzed and compared the biochemistry, autoimmune antibodies, and liver biopsy results of 129 autoimmune hepatic disease cases retrospectively, using the international criteria to see which could be diagnosed as AIH/PBC overlap syndrome.

RESULTS

Our 35 AIH+PBC overlap syndrome patients were mainly women, with a sex ratio of 1 female: 10 male, and a median age of 50.79+/-11.27 (20 to 70 years old). They had AIH characteristics such as flare of ALT, AST and elevated immunoglobulin G (IgG), gamma-immunoglobulin. There were also antinuclear antibodies (74.3%); moderate or severe periportal or periseptal lymphocytic infiltration, piecemeal necrosis, and florid bile duct lesions, high serum levels of ALP, presence of mitochondrial antibodies (68.6%) and M2 antibodies (45.7%), and features of PBC.

CONCLUSIONS

AIH+PBC overlap syndrome is not rare. It should be diagnosed in time and to find effective treatments for it.

摘要

目的

了解自身免疫性肝炎-原发性胆汁性肝硬化（AIH+PBC）重叠综合征的临床特征。

方法

回顾性分析并比较129例自身免疫性肝病患者的生化指标、自身抗体及肝活检结果，采用国际标准判断哪些可诊断为AIH/PBC重叠综合征。

结果

35例AIH+PBC重叠综合征患者以女性为主，男女比例为1∶10，中位年龄为50.79±11.27岁（20至70岁）。他们具有AIH的特征，如谷丙转氨酶（ALT）、谷草转氨酶（AST）升高及免疫球蛋白G（IgG）、γ-免疫球蛋白升高。还存在抗核抗体（74.3%）；中度或重度汇管区或间隔周围淋巴细胞浸润、桥接坏死及明显的胆管病变，血清碱性磷酸酶（ALP）水平升高，存在线粒体抗体（68.6%）及M2抗体（45.7%），具有PBC的特征。