原发性胆汁性肝硬化与自身免疫性胆管炎的临床特征和HLA-DRBl等位基因分析  

Clinical pathology and HLA-DRB1 allele associations of primary biliary cirrhosis and autoimmune cholangitis in Han Chinese

作　　者：陈嵩[1] 朱刚剑[2] 邓国宏[1] 朱妍[1] 

机构地区：[1]第三军医大学西南医院全军感染病研究所,重庆市400038 [2]荆州市第二人民医院感染科,湖北省荆州市434000

出　　处：《世界华人消化杂志》2005年第20期2450-2454,共5页World Chinese Journal of Digestology

摘　　要：目的:分析原发性胆汁性肝硬化(PBC)和自身免疫性胆管炎(AIC)患者的临床表现、肝组织病理学特征及其与HLA-DRB1等位基因的相关性.方法:回顾性分析PBC患者94例,筛选出13例AIC、9例PBC和自身免疫性肝炎(AIH)重叠综合征(PBC/AIH重叠综合征),分析AIC、PBC/AIH重叠综合征和单纯PBC患者的临床表现和肝组织病理学特征,采用序列特异性多聚酶链反应(PCR-SSP)进行HLA-DRB1等位基因分析.结果:AIC和单纯PBC比较,在年龄、性别、血生化及肝组织病理学上不能区分二者,但是AIC患者治疗前AIH计分(8.8±0.9vs4.6±0.8,t=17.45,P<0.01)、ANA和/或SMA阳性率(84.6%vs22.2%,c2=17.0039,P<0.01)明显高于单纯PBC.AIC患者血清ALT、AST、IgG明显低于PBC/AIH重叠综合征患者.PBC患者的HLA-DRBl*08基因频率比健康对照组的显著增高(9.7%vs3.3%,RR=4.42,P<0.05),而比AIC患者有增高的趋势.结论:AIC可能仅是PBC的一种变异形式,中国汉族PBC的发病与HLA-DR8相关.AIM： To analyze the clinical and pathological characteristics of both primary biliary cirrhosis （PBC） and autoimmune cholangitis （AIC）, and to explore their associations with HLA-DRB1 alleles in Han Chinese. METHODS： Of 94 patients diagnosed with PBC, 12 cases of AIC and 9 cases of overlap syndrome of PBC and autoimmune hepatitis （AIH） were identified. The clinical manifestations and pathological changes exhibited in the liver biopsies from AIC, pure PBC and overlap syndrome were comparatively analyzed. The frequencies of HLA- DRB1 alleles were determined in AIC and PBC patients and healthy controls by polymerase chain reaction with sequence specific primers. RESULTS： The age, sex, clinical manifestations, bio-chemical markers and histological finding.s were not significantly different between the AIC and pure PBC patients. However, the score for AIH and the positive rate of serum antinuclear antibody and/or smooth muscle antibody were higher in patients （8.8± 0.9 vs 4.6±0.8, t = 17.45, P 〈0.01; 84.6% vs 22.2%, x^2 = 17.003 9, P 〈0.01）. The serum levels of alanine aminotransferase （ALT）, aspartate transaminase （AST） and immunoglobulin G in AIC patients were markedly lower than those in the overlap syndrome patients （t = 3.44, P 〈0.01; t = 2.83, P 〈0.01; t = 2.18, P 〈0.05）. Higher allele frequency of HLA-DRBI＊08 was found in pure PBC patients, versus controls （9.7% vs 3.3%, RR = 4.42, P 〈0.05）, and the allele frequency of HLA- DRBI＊08 tended to increase in pure PBC patients. CONCLUSION： Except the pretreatment score for AIH and the types of immunoserological marker, there were no differences between AIC and pure PBC. AIC may only be a variant pattern of PBC, rather than a separate disease.

关 键 词：肝硬化 胆汁性 肝炎 自身免疫性 胆管炎 重叠综合征 HLA-DRBI等位基因 原发性胆汁性肝硬化 自身免疫性胆管炎 基因分析 

分 类 号：R575.2[医药卫生—消化系统] R575.6[医药卫生—内科学]