原发性胆汁性胆管炎和原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征患者临床与肝组织病理学特点分析

Clinical feature and biochemical response to immunosuppressive therapy in patients with primary biliary cholangitis and PBC-autoimmune hepatitis overlapping syndrome

目的比较分析原发性胆汁性胆管炎(PBC)和PBC-自身免疫性肝炎(AIH)重叠综合征(OS)患者临床特征。方法2017年5月~2023年2月南通大学附属南通第三医院诊治的PBC患者64例和PBC-AIH OS患者27例。常规进行肝穿刺检查,分别给予熊去氧胆酸(UDCA)或UDCA联合泼尼松(龙)治疗,观察1年。结果PBC-AIH OS患者血清AST、球蛋白和IgG水平分别为103.5(74.5,213.3)U/L、34.2±7.3g/L和19.1±8.1g/L,显著高于PBC患者【分别为71.0(32.0,119.0)U/L、30.2±6.2g/L和14.7±5.3g/L,P<0.05】;PBC-AIH OS患者肝组织炎症分级、纤维化分期、界面性肝炎、融合坏死和汇管区炎症等方面均显著重于PBC患者(P<0.05);在治疗1年末,64例PBC患者生化学完全应答、部分应答和无应答率分别为56.3%、26.7%和17.2%,而27例PBC-AIH OS患者则分别为44.4%、37.0%和18.5%。结论PBC和PBC-AIH OS主要发生在中老年女性,两种疾病临床表现和特征性血清自身抗体阳性率无显著性差异,但PBCAIH OS患者肝组织病理学病变较单纯PBC患者更重,治疗应答相对差,需要认真管理。

Objective The clinical feature and biochemical response to immunosuppressive therapy were compared between patients with primary biliary cholangitis(PBC)and PBC-autoimmune hepatitis(AIH)overlap syndrome(OS).Methods 64 patients with PBC and 27 patients with PBC-AIH OS were encountered in our hospital between May 2017 and February 2023,and all undergone liver biopsies.The patients in the two groups were treated by ursodeoxycholic acid(UDCA)or UDCA and prednisone combination,and response were assessed by one year.Results Serum AST,globulin and IgG levels in patients with PBC-AIH OS were 103.5(74.5,213.3)U/L,34.2±7.3g/L and 19.1±8.1g/L,all significantly higher than[71.0(32.0,119.0)U/L,30.2±6.2g/L and 14.7±5.3g/L,respectively,P<0.05]in patients with PBC;histo-patholocially,hepatic activity index,fibrosis staging,interfacial inflammation,fusion necrosis and portal inflammation in patients with PBC-AIH OS were much more severe than in those with PBC(P<0.05);by one year,complete,partial and non-biochemical response were 56.3%,26.7%and 17.2%in 64 patients with PBC,while they were 44.4%,37.0%and 18.5%in 27 patients with PBC-AIH OS.Conclusion PBC and PBC-AIH OS predominantly involve in middle-aged and elderly women,and there is little discrepancy in clinical manifestations and characteristic serum autoimmune antibodies between the two groups,while the intrahepatic histo-pathologic damage were obviously severe in patients with PBC-AIH OS,with relatively poor response rate to immunosuppressive therapy,which warrants close surveillance in clinical practice.