加强对自身免疫性肝病三联重叠综合征的认识  

Diagnosis and treatment of trigeminy overlap syndrome of autoimmune liver disease

作　　者：高素纪 徐芸[1] 保洁 刘象 刘香漫 华静[1] 

机构地区：[1]郑州大学第一附属医院消化内科,河南省郑州市450052

出　　处：《世界华人消化杂志》2016年第2期293-299,共7页World Chinese Journal of Digestology

摘　　要：目的:增强对自身免疫性肝病中三联重叠综合征(autoimmune hepatitis-primary biliary cirrhosis-primary sclerosing cholangitis overlap syndromes,AIH-PBC-PSC OS)的认识,提高对自身免疫性肝病中AIH-PBC-PSC OS的诊断及治疗水平.方法:经肝活检确诊的7例AIH-PBC-PSC OS患者,收集其临床特征、生化指标、自身抗体、影像学及肝组织学指标,7例患者给予口服熊去氧胆酸(ursodeoxycholic acid,UDCA)胶囊[13-15 mg/(kg·d)]、泼尼松(30 mg/d,每周递减5 mg,至10 mg/d维持)和硫唑嘌呤(azathioprine,AZA)(50 mg/d),观察疗效.结果:谷丙转氨酶(alanine aminotransferase,ALT)、谷草转氨酶(aspartate aminotransferase,AST)、γ-谷氨酰转肽酶(gamma glutamyl transpeptidase,GGT)、碱性磷酸酶(alkaline phosphatase,ALP)治疗后比基线水平降低,总胆红素(total bilirubin,TBIL)治疗后较基线水平升高.A L P比较差异有统计学意义(P<0.05),ALT、AST、GGT、球蛋白(globulin,GLOB)、TBIL、Ig G、Ig M比较差异均无统计学意义(P>0.05),7例患者均肝穿活检,病理结果均显示AIH、PBC及PSC的特征性表现.随访中1例行肝移植,术后肝功能正常,4例去世(3例死于肝衰竭,1例死于消化道出血).结论:自身免疫性肝病血清特异性抗体及特征性组织学、影像学是诊断自身免疫性肝病的金标准.AIH-PBC-PSC OS患者药物治疗效果差,出现症状后短期死亡率高.肝移植仍是治疗终末期O S患者的唯一有效方法.AIM： To investigate the diagnosis and treatmentof trigeminy overlap syndrome [autoimmune hepatitis-primary biliary cirrhosis-primary sclerosing cholangitis overlap syndrome （AIH- PBC-PSC OS）] of autoimmune liver disease （AILD）. METHODS： All data for seven patients diagnosed with AIH-PBC-PSC OS by liver biopsy, including clinical characteristics and biochemistry indexes, autoantibodies, imaging findings and liver histology, were analyzed. Ursodeoxycholic acid （UDCA） [13-15 mg/（kg,d）], prednisone （30 mg/d, diminished by 5 mg/ qw, until 10 rag/d） and azathioprine （AZA） （50 rag/d） were given to these patients. RESULTS： Over levels of alanine 12 mo of observation, the aminotransferase （ALT）, aspartate aminotransferase （AST）, 7-glutamyl transpeptidase （GGT）, and alkaline phosphatase （ALP） in all the 7 patients decreased, and the levels of TBIL increased. Compared with baseline value, there was a significant decrease in ALP （P 〈 0.05）. However, there were no significant differences in other parameters （P 〉 0.05）. Pathological results showed the characteristics of AIH, PBC and PSC. Liver transplantation was performed in one patient, and the hepatic function of the patient was normal after operation. Four patients died （one died of hepatic failure and one died of gastrointestinal bleeding） during follow-up. CONCLUSION： The diagnosis of AILD is based mainly on serologic tests and characteristic histological features. The short-term mortality of AIH-PBC-PSC OS is high. The effect of medical treatment for patients with AIH-PBC- PSC OS is poor. Liver transplantation is theonly option with known therapeutic benefit for end-stage patients.

关 键 词：自身免疫性肝病 重叠综合征 诊断 治疗 

分 类 号：R575[医药卫生—消化系统]