抗线粒体抗体阴性原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征的临床病理分析

Clinicopathological analysis of anti-mitochondrial antibody negative primary biliary cholangitis-autoimmune hepatitis overlap syndrome

目的分析抗线粒体抗体(AMA)阴性的原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征(PBC-AIHOS)患者与AMA阳性PBC-AIHOS患者的临床病理特点。方法连续纳入2017.06—2018.04确诊为PBC-AIHOS的患者共74例,其中40例为AMA阴性(阴性组),34例为AMA阳性(阳性组),分别比较两组患者的临床表现、血清生物化学指标、免疫学指标及肝活组织检查病理学资料。分别用Mann-Whitney U检验和χ^2检验进行统计学分析。结果AMA阴性组、AMA阳性组PBC-AIHOS患者在性别、年龄、临床表现及主要肝功能指标(丙氨酸氨基转移酶、天冬氨酸氨基转移酶、碱性磷酸酶、γ-谷氨酰转移酶、总胆红素、直接胆红素)差异均无统计学意义(P>0.05);阴性组血清中IgM水平(1.68±0.87)g/L较阳性组(3.77±2.88)g/L明显下降(P<0.05);阴性组ANA和gp-210抗体阳性率较阳性组低,差异有统计学意义(P<0.05);肝组织病理方面,两组比较炎症和纤维化分期差异均无统计学意义(P>0.05),阴性组较阳性组胆管损伤更加显著(P<0.05)。结论AMA阴性的PBC-AIHOS血清IgM水平较低,往往有免疫学抗体阴性,更易出现肝组织学中胆管损伤。应尽早进行肝穿刺活组织检查,早期诊断,早期治疗。

Objective To explore the clinicopathological features of anti-mitochondrial antibody(AMA)negative and positive patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome(PBC-AIH OS).Methods Seventy-four cases diagnosed as PBC-AIH OS from June 2017 to April 2018 were enrolled in this study.Among them,forty cases were AMA negative(negative group)and thirty-four cases were AMA positive(positive group).The clinical manifestations,serum biochemical indexes,immunological indexes and histological data of the negative group were compared with the positive group.Mann-Whitney U test and the χ^2 test were used for statistical analysis.Results There was no significant difference in sex,age,clinical manifestations and major liver function indexes(ALT,AST,ALP,GGT,TB,DB)between the negative group and positive group(P>0.05).The level of IgM in the negative group(1.68±0.87)was significantly lower than positive group(3.77±2.88)(P<0.05).The positive rates of antinuclear antibodies(ANA)and gp-210 antibodies was lower than positive group(P<0.05).There were no significant differences in the stages of inflammation and fibrosis between the two groups(P>0.05),and the bile duct injury was more significant in the negative than positive group(P<0.05).Conclusion The serum IgM level of AMA-negative PBC-AIH OS is low,and immunological antibody is often negative,which makes bile duct injury apparent in liver histology.A liver biopsy should be carried out as soon as possible for early diagnosis and treatment.