122例自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征的临床特点分析  

Characteristic analysis on 122 patient with autoimmune hepatitis-primary biliary cholangitis overlap syndrome

作　　者：苏杞敏[1] 王轶[1] 陈慧婷[1] 淦伟强[2] SU Qi-min;WANG Yi;CHEN Hui-ting;GAN Wei-qiang(Department of Infectioas Diseases,the First People's Hospital of Guangzhou,Guangzhou,Guangdong 510180;Department of Infectious Diseases,the Third Affiliated Hospital of Zhongshan University,Guangzhou,Guangdong 510630,China)

机构地区：[1]广州市第一人民医院感染科,广东广州510180 [2]中山大学附属第三医院感染科,广东广州510630

出　　处：《热带医学杂志》2021年第7期916-920,F0003,共6页Journal of Tropical Medicine

基　　金：广东省医学科学技术研究基金(A20200411);广州市科技计划项目(201904010132)。

摘　　要：目的分析自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征(AIH-PBC OS)患者的临床特征,提高对该病的认识,为临床诊断与治疗提供帮助。方法收集2009年1月-2019年1月期间中山大学附属第三医院就诊的AIH-PBC OS患者122例,对患者的临床、血清学、影像学和病理学特点,伴发肝外自身免疫性疾病等情况进行回顾性分析。结果122例AIH-PBC OS患者男女比例为1∶12,平均发病年龄(48.8±11.4)岁,确诊年龄(51.2±10.2)岁,病程(2.0±3.7)年。诊断为慢性肝炎50例(41.0%),肝硬化69例(56.6%)(其中代偿性肝硬化18例,失代偿性肝硬化51例),急性肝衰竭2例(1.6%)。28例(23.0%)患者合并肝外自身免疫性疾病,以自身免疫性甲状腺疾病、干燥综合征和类风湿关节炎最常见。血清丙氨酸氨基转移酶(ALT)、天门冬氨酸氨基转移酶(AST)、碱性磷酸酶(ALP)、谷氨酰转肽酶(GGT)、总胆红素(TBIL)、直接胆红素(DBIL)、总胆汁酸(TBA)、球蛋白(GLB)、总胆固醇(CHOL)、低密度脂蛋白胆固醇(LDL-C)水平均升高,血红蛋白(HGB)和白蛋白(ALB)明显下降。57例(46.7%)血清IgG升高,58例(47.6%)IgM水平升高。自身抗体包括抗核抗体(ANA)阳性率、抗线粒体抗体(AMA)和(或)抗线粒体抗体M2型抗体(AMA-M2)阳性率、抗平滑肌抗体(SMA)阳性率、gp210阳性率、sp100阳性率分别为94.0%、79.5%、3.2%、18.0%、8.2%。影像学提示,肝硬化51例(41.8%),门静脉高压32例(26.2%),脾大76例(62.3%),腹腔积液31例(25.4%)。肝脏组织病理学显示,AIH-PBC OS患者可见肝小叶界面炎,汇管区淋巴细胞和浆细胞浸润,并伴有不同程度小胆管病变,73.7%同时伴有AIH-PBC特征。结论AIH-PBC OS临床表现和生化指标更类似于PBC,但免疫学和病理结果更类似于AIH;同时伴有HGB下降,血脂代谢障碍,且易于并发肝外自身免疫性疾病。Objective To analyze the clinical characteristics of autoimmune hepatitis(AIH)-primary biliary cholangitis(PBC)overlap syndrome(AIH-PBC OS),and improve clinical diagnosis and treatment.Methods A retrospectively analysis was performed for the clinical data,biochemical and immunological parameters,imaging findings,liver histopathological features and incidence of extrahepatic autoimmune disease of 122 patients with AIH-PBC OS from January 2009 to January 2019.Results The ratio of male to female of 122 patients with AIH-PBC was 1∶12;the average age was(48.8±11.4)years old;the diagnosis age was(51.2±10.2)years old,and the duration of disease was(2.0±3.7)years.The patient diagnosed chronic hepatitis was about 41.0%;cirrhosis was about 56.6%,which included compensated liver cirrhosis(14.7%)and decompensated cirrhosis(41.8%);acute hepatic failure was about 1.6%.About 23.0% of patients had extrahepatic autoimmune diseases,such as Sjogren’s syndrome,thyroid disease,and heumatoid arthritis were all more common among those with AIH-PBC.Serum levels of alanine aminotransferase(ALT),aspartate transaminase(AST),alkaline phosphatase(ALP),gamma-glutamyl transpeptidase(GGT),total bilirubin(TBIL),direct bilirubin(DBIL),totla bile acid(TBA),globulin(GLB),cholesterol(CHOL),triglyceride(TG),low density lipoprotien cholesterol(LDL-C)were increased in the patients,and hemoglobin(HGB)and albumin(ALB)were diclined.IgG(46.7%)and IgM(47.6%)levels were increased.The positive rates of anti-nuclear antibodies(ANA),anti-mitochondrial antibody(AMA)and/or anti-mitochondrial antibody M2(AMA-M2),anti-smooth muscle antibodies(SMA),gp210 and sp100 were 94.0%,79.5%,3.2%,18.0% and 8.2%,respectively.Cirrhosis was diagnosed in 51 patients(41.8%)by imaging;portal hypertension was found in 32 patients(26.2%);splenomegalia was found in 76 patients(62.3%)and seroperitoneum was found in 31 patients(25.4%).The liver histopathology shows hepatocellular damage such as lobular interface,inflammatory cell infiltrating in portal duct areas and different deg

关 键 词：胆汁性 肝炎 自身免疫性 重叠综合征 

分 类 号：R575[医药卫生—消化系统]