原发性胆汁性肝硬化/自身免疫性肝炎重叠综合征28例临床和病理学分析

摘要

Objective To summarize the clinical,biochemical and histological characteristics of patients with overlap syndrome of primary biliary cirrhosis(PBC)and autoimmune hepatitis(AIH). Methods According to PBC diagnosis published by the American Association for the Study of Liver Disease in 2008 and the Criteria for Di-agnosis of AIH simplified scoring systems,we retrospectively analyzed the clinical,biochemical and histological characteristics of 28 patients diagnosed with PBC-AIH overlap syndrome. Results Serum alanine transaminase (ALT),aspartate transminase(AST),alkanline phosphatase(ALP), glutamine transpeptidase(γ-GT), total bilirubin (TBIL)and direct bilirub(DBIL)levels in patients with PBC-AIH overlap syndrome were 154.93±28.68U/L,185.21± 39.25U/L,283.86 ±30.99U/L,352.36 ±71.15U/L,34.15 ±7.79μmol/L,11.15 ±0.86μmol/L,respectively,which were obvi-ously higher than in normal persons (ALT,AST,ALP,γ-GT,TBIL and DBIL were 17.8±1.60U/L,20.29±1.02U/L,67. 89±3.31U/L,20.51±3.33U/L,11.15±0.86μmol/L and 3.35±0.28μmol/L,respectively,P<0.05);Serum levels of immuno-golobulin G(IgG),immunogolobulin M(IgM)in overlap syndrome patients were high;The positive rates of antinuclear antibody(ANA) was 78.6%,and anti-mitochondrial antibody(AMA)-M2 in overlap syndrome were 71.4%;Histologi-cally, the livers in the overlap syndrome group showed combined features of interface hepatitis and bile duct le-sion. Conclusions PBC-AIH overlap syndrome is more common in middle-aged women. It has clinical,biochemi-cal,and histological characteristics of both PBC and AIH.%　　目的总结原发性胆汁性肝硬化(PBC)-自身免疫性肝炎(AIH)重叠综合征患者临床及组织病理学特点.方法采用2009年美国肝病学会修订的PBC诊断标准和2008年简化的AIH诊断标准,对28例PBC-AIH重叠综合征患者的临床及病理学资料进行回顾性分析.结果28例PBC-AIH重叠综合征患者ALT为154.93±28.68U/L,AST 为185.21±39.25U/L,ALP 为283.86±30.99U/L,γ-GT 为352.36±71.15U/L,TBIL 为34.15±7.79μmol/L,DBIL为11.15±0.86μmol/L,均显著高于正常人(ALT为17.8±1.60U/L,AST为20.29±1.02U/L,ALP为67.89±3.31U/L,γ-GT为20.51±3.33U/L,TBIL为11.15±0.86μmol/L,DBIL为3.35±0.28μmol/L,P<0.05)；血清IgG和IgM升高,自身抗体中ANA(78.6%)和AMA-M2(71.4%)阳性率较高；肝穿组织可见界面性肝炎和小胆管损伤.结论 PBC-AIH重叠综合征多见于女性,在临床及组织病理学上兼有PBC和AIH的双重特点.