自身免疫性肝炎／原发性胆汁性肝硬化重叠综合征患者临床特征分析

摘要

目的：比较自身免疫性肝炎（AIH）和胆汁性肝硬化（PBC）与 AIH／PBC重叠综合征患者的生物化学、免疫学指标及病理组织学特点，为制定 AIH／PBC重叠综合征临床诊断的参考标准及合理的治疗方案提供依据。方法：选取135例自身免疫性肝病患者，其中 AIH 患者49例、PBC 患者43例、AIH／PBC 重叠综合征患者43例，检测各类型患者的生物化学、免疫学和组织病理学等指标，并对 AIH／PBC患者行不同的治疗方案，分别给予患者熊去氧胆酸、泼尼松及熊去氧胆酸和泼尼松联合治疗，评价各治疗方案的有效性。结果：生化指标， AIH／PBC组患者γ-谷氨酰转移酶（GGT）、碱性磷酸酶（ALP）活性和 IgM 水平显著高于 AIH 组（P＜0.05）， AIH／PBC组患者的丙氨酸转氨酶（ALT）、谷草转氨酶（AST）活性和 IgG水平显著高于PBC组（P＜0.05）。自身抗体指标，与 AIH组比较，AIH／PBC组患者抗线粒体抗体（AMA）和 M2型线粒体抗体（AMA M2）检出率增高（P＜0.05）；与PBC组比较，AIH／PBC组患者抗核抗体（ANA）和抗平滑肌抗体（SMA）检出率增高（P＜0.05）。病理学检查，AIH／PBC组患者存在高发生率的碎屑样坏死（100.00％）、肝细胞玫瑰花环样改变（83.72％）及胆管病变（69.77％）。对 AIH／PBC患者实施联合用药组有效率为85.7％，显著高于各种单独用药组（P＜0.05）。结论：AIH／PBC重叠综合征患者的生物化学、免疫学指标和病理组织学变化兼有 AIH 和 PBC患者的特点，因此可以作为其诊断依据。泼尼松联合熊去氧胆酸治疗 AIH／PBC综合征取得了较好的疗效，值得临床推广。%Objective To compare the biochemical and immunological parameters and histopathological characteristics in the patients with autoimmune hepatitis (AIH),biliary cirrhosis (PBC)and AIH/PBC overlap syndrome,and to provide basis for working out the reference standard of clinical diagnosis and reasonable treatment.Methods 135 cases of autoimmune liver disease patients were selected,including 49 cases of AIH patients,43 cases of PBC patients, and 43 cases of AIH/PBC overlap syndrome patients. The biochemical, immunological parameters and histopathological changes of the patients were detected. The patients with AIH/PBC overlap syndrome were treated with different therapy methods including ursodesoxycholic acid (UDCA ) treatment,prednisone treatment and combination treatment with UDCA and prednisone,and the effectiveness of different treatment programs were evaluated.Results The activities of GGT and ALP and IgM level of the patients in the AIH/PBC group were significantly higher than those in AIH group,there were significant differences (P<0.05);the activities of ALT and AST and IgG level of the patients in AIH/PBC group was significantly higher than those in PBC group,there were significant differences (P<0.05).The detection rates of AMA and AMA M2 of the patients in AIH/PBC group were higher than those in AIH group, there were significant differences (P<0.05);the detection rates of ANA and SMA of the patients in AIH/PBC group were higher than those in PBC group,there were significant differences (P<0.05).There were high incidence of piecemeal necrosis (100.0%), liver cell rosette-like changes (83.72%)and bile duct lesions (69.77%)of the patients in AIH/PBC group.The effective percentage in combination therapy group was 85.7% which was significantly higher than those in various drug alone groups (P< 0.05 ). Conclusion The changes of biochemical and immunological indicators and pathological features of the patients with AIH/PBC overlap syndrome are in combination with the particular indicators of AIH and PBC which would provide the diagnostic basis for AIH/PBC overlap syndrome. The combined therapy for AIH/PBC overlap syndrome is effective and should be popularized.