AIH与AIH-PBC重叠综合征的临床和病理特征  

Clinical and pathological features of AIH versus AIH- PBC overlap syndrome

作　　者：谢月萍[1] 李刚平[2] 郭晖[3] 杨玲[2] 宋宇虎[2] 叶进[2] 

机构地区：[1]南宁市第一人民医院老年科,广西壮族自治区南宁市530022 [2]华中科技大学同济医学院附属协和医院消化内科,湖北省武汉市430022 [3]华中科技大学同济医学院附属同济医院器官移植研究所,湖北省武汉市430030

出　　处：《世界华人消化杂志》2013年第32期3473-3478,共6页World Chinese Journal of Digestology

摘　　要：目的:比较自身免疫性肝炎(autoimmune hepatitis,AIH)与自身免疫性肝炎-原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)重叠综合征(AIH-PBC overlap syndrome)的临床和病理特点,以提高诊断AIH-PBC重叠综合征的水平.方法:收集我院2005-01-01/2010-12-31共50例诊断为AIH及AIH-PBC重叠综合征患者的临床及病理资料,分析并比较两组的不同特点.结果:AIH组30例,AIH-PBC重叠综合征组20例:其中AIH组行肝活检17例,占56.7%,AIHPBC重叠综合征12例,占60%.两组的丙氨酸氨基转移酶(alanine aminotransferase,ALT)和门冬氨酸氨基转移酶(aspartate aminotransferase,AST)无统计学差异.AIH-PBC重叠综合征组碱性磷酸酶(alkaline phosphatase,ALP)和-谷氨酰转肽酶(-glutamyl transpeptidase,-GT)及血清总胆红素(total bilirubin,TBIL)均显著高于AIH组水平(P<0.01).AIH组病理特点为汇管区大量淋巴细胞浸润、界板性炎症、肝小叶内少数灶状和碎屑样肝细胞坏死,但小叶间胆管未见明显异常;AIH-PBC重叠综合征组病理特点:除具备AIH病理特点外,还可见汇管区小叶间胆管上皮内淋巴细胞浸润,胆管上皮细胞空泡变,小叶间胆管结构萎缩,少数病例可见汇管区内纤维组织明显增生和少数假胆管增生.结论:AIH-PBC重叠综合征组的ALP和-GT及血清总胆红素水平较AIH组高;病理特点上AIH组表现为汇管区大量淋巴细胞浸润、界板性肝炎;AIH-PBC重叠综合征组除具备AIH组织学特点外还有不同程度的胆管损伤.AIM： To compare the clinical and pathological characteristics of patients with autoimmune hep- atitis （AIH） and those with overlap syndrome of primary biliary cirrhosis （PBC） and autoimmune hepatitis （AIH-PBC overlap syndrome）. METHODS： Thirty patients with AIH and twen- ty patients with AIH-PBC overlap syndrome who were diagnosed at our hospital from Janu- ary 2005 to December 2010 were involved in this study. The clinical features, biochemical markersand histological characteristics were retrospec- tively analyzed and compared between the two groups. RESULTS： Seventeen （56.7%） AIH patients and twelve （60%） AIH-PBC overlap syndrome pa- tients accepted liver biopsy. There were no sta- tistical differences in the levels of alanine amino- transferase （ALT） and aspartate aminotransfer- ase （AST） between AIH patients and AIH-PBC overlap syndrome patients （P = 0.259, 0.889, both P 〉 0.05）. AIH-PBC overlap syndrome patients had significantly higher levels of alkaline phos- phatase （ALP）, l-glutamyl transpeptidase （1-GT） and total bilirubin （TBIL） than AIH patients （all P 〈 0.01）. The pathological features of AIH pa- tients included remarkable portal mononuclear cell infiltration, interface hepatitis, few focal and piecemeal necrosis in the hepatic lobule, and no abnormal findings in interlobular bile ducts. In AIH-PBC overlap syndrome patients, mono- nuclear cell infiltration in the portal interlobular bile duct, bile duct epithelial cell cavitation and interlobular bile duct structure atrophy as well as all pathological features of AIH patients were observed. Some patients had obvious portal fi- ber tissue hyperplasia and pseudo-bile duct pro- liferation.CONCLUSION： Patients with AIH-PBC overlap syndrome have higher levels of ALP, y-GT and TBIL than those with AIH. In addition to patho- logical features of AIH, AIH-PBC overlap syn- drome also shows varying degrees of bile duct damage, such as remarkable portal mononuclear cell infiltration and interface hepat

关 键 词：自身免疫性肝炎 自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征 临床特点 病理特点 不同比较 

分 类 号：R575.1[医药卫生—消化系统]