伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征

Clinical characteristics analysis of primary biliary cholangitis with features of autoimmune hepatitis

目的分析伴自身免疫性肝炎(AIH)特征的原发性胆汁性胆管炎(PBC)患者的临床特征。方法纳入2008年12月-2018年12月空军军医大学附属西京医院消化内科确诊的PBC患者461例,根据PBC-AIH重叠综合征(PBC-AIH OS)、AIH-PBC及PBC的诊断标准分为PBC-AIH OS组(n=50)、AIH-PBC组(n=178)与单纯PBC组(n=233),分析各组一般临床症状(黄疸、乏力、瘙痒等)、生化免疫指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、免疫球蛋白G/M(IgG、IgM)、总/直接胆红素(TBIL、DBIL)、白/球蛋白(ALB、GLB)]、自身抗体[抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(ASMA)]、肝脏炎症纤维化分级分期、基于4因子的纤维化指数(FIB-4)和天门冬氨酸氨基转移酶/血小板比值指数(APRI),以及5年不良事件累积发生率等。结果 AIH-PBC组及单纯PBC组黄疸、纳差发生率均明显低于PBC-AIH OS组(P<0.05)。各组ALT、AST、TBIL、IgG、IgM、GLB水平差异有统计学意义(P<0.05),多以AIH-PBC组及PBC-AIH OS组较高。自身抗体检测结果显示,AIH-PBC组SSA、Ro-52抗体检出率明显高于单纯PBC组,ASMA、可溶性肝抗原/肝胰抗原抗体(SLA/LP)、SSB检出率明显低于PBC-AIH OS组(P<0.05)。AIH-PBC组IgG、IgM水平高于单纯PBC组(P<0.05)。AIH-PBC组和PBC-AIH OS组肝组织病理分级分期及FIB-4、APRI指数明显高于单纯PBC组(P<0.05)。5年累积不良事件发生率PBC-AIH OS组明显高于AIH-PBC组,AIH-PBC组明显高于单纯PBC组(P<0.05)。结论 AIH-PBC患者在临床表现上与PBC-AIH OS及单纯PBC患者有较大差异,长期预后较单纯PBC患者差。

Objective To analyze the clinical characteristics of 178 primary biliary cholangitis (PBC) patients with features of autoimmune hepatitis (AIH).Methods Four hundred and sixty-one PBC patients diagnosed in the Department of Gastroenterology,Xijing Hospital Affiliated to Air Force Medical University from December 2008 to December 2018 were included in this study.According to the diagnostic criteria of PBC-AIH,AIH-PBC and PBC,they were divided into PBC-AIH OS group (n=50),AIH-PBC group (n=178) and pure PBC group (n=233).Comparison of general clinical symptoms of three groups (jaundice,fatigue,pruritus,etc.);biochemical and immunological indices[alanine aminotransferase (ALT),aspartate aminotransferase (AST),alkaline phosphatase (ALP),gamma glutamyltranspeptidase (GGT),immunoglobulin G/M (IgG,IgM),total/direct bilirubin (TBIL,DBIL),albumin/globulin (ALB,GLB)];autoantibodies[antinuclear antibody (ANA),antimitochondrial antibody (AMA),anti-smooth muscle antibody (ASMA)];stage and grade of the inflammation and fibrosis of the liver,noninvasive fibrosis score of fibrosis index based on the four factors (FIB-4) and aspartate aminotransferase/platelet ratio index (APRI);5-year cumulative incidence of adverse events.Results The incidence of jaundice and inappetence in AIH-PBC group and PBC group was significantly lower than that in PBC-AIH OS group (P<0.05).The differences of ALT,AST,TBIL,IgG,IgM and GLB between the groups were statistically significant (P<0.05),and AIH-PBC group and PBC-AIH OS group were mostly higher than pure PBC group.Autoantibody detection showed that the positivity rates of SSA and Ro-52 antibodies in AIH-PBC group were significantly higher than those in pure PBC group,while the positivity rates of ASMA,soluble liver antigen/liver pancreatic antigen antibody (SLA/LP) and SSB were significantly lower than those in PBC-AIH OS group (P<0.05).The IgG and IgM of AIH-PBC group were significantly higher than those of pure PBC group (P<0.05).The liver histopathological stage,FIB-4 and APRI indexes of AIH-PBC group and PBC-AIH OS group were significantly higher than those of pure PBC group (P<0.05).The 5-year cumulative adverse event incidence of PCB-AIH OS group was significantly higher than that of AIH-PBC group,and that of AIH-PBC group was significantly higher than that of pure PBC group (P<0.05).Conclusions The clinical manifestations of AIH-PBC patients are significantly different from those of PBC-AIH OS and pure PBC patients,and their long-term prognosis is worse than that of pure PBC patients.