125例自身免疫性肝病的临床特点分析

发布时间：2018-06-08 01:51

本文选题：自身免疫性肝病 + 自身免疫性肝炎　；参考：《宁夏医科大学》2017年硕士论文

【摘要】：目的分析自身免疫性肝病患者的临床特点,为自身免疫性肝病的诊断和鉴别诊断提供更多依据。方法收集2010年1月至2016年9月宁夏医科大学总医院确诊自身免疫性肝病(AILD)的患者共125例,包括38例自身免疫性肝炎(AIH)、82例原发性胆汁性胆管炎(PBC)、5例原发性硬化性胆管炎(PSC),分析总结患者的一般情况、临床表现、合并症情况、实验室检查、影像学及组织病理学检查的特点。结果 AILD好发于中老年女性,平均年龄为(58.00±13.26)岁,男女比例为1:4.95。各组在性别构成及年龄上无差异。各组临床表现无特异性。AIH组生化指标以ALT、AST升高为主,PBC组及PSC组以ALP、GGT、TBIL升高为主,三组ALB水平均降低,但AIH组GLB水平高于PBC组及PSC组(P0.05)。AIH组Ig G水平高于PBC组(P0.05),PBC组Ig M水平高于AIH组(P0.05),PSC组Ig G和Ig M水平无明显变化。AIH组ANA阳性率与PBC组比较无差别(P0.05),但在ANA分型上,AIH组以斑点型(42.42%)为主,PBC组以胞浆颗粒型(50.75%)为主;AIH的ASMA阳性率高于PBC组(P0.05)。LKM-1、SLA/LP及LC-1是AIH比较特异性的自身抗体,AMA/AMA-M2是PBC特异性的自身抗体,而PSC未发现特异性的自身抗体。腹部彩超、腹部CT在检测AIH和PBC患者肝硬化、腹腔积液的病变上比较无差异(P0.05),在PSC中未发现肝硬化及腹腔积液病变,腹部CT表现为肝内外胆管的扩张。AIH和PBC在MRCP/ERCP中未发现胆管病变,而PSC表现为肝内外胆管枯树枝样改变、僵硬和节段性狭窄。AIH组织学表现为肝细胞变性、坏死,PBC为小胆管增生及胆汁淤积改变。结论 1.AILD好发于中老年女性;2.AILD在临床表现上缺乏特异性;3.AIH的临床特点为ALT、AST、Ig G水平升高,常见的自身抗体有ANA、ASMA,特异性的自身抗体有LKM-1、SLA/LP、LC-1,组织学表现为肝脏实质损害;4.PBC的临床特点为ALP、GGT、TBIL和Ig M水平升高,特异性的自身抗体为AMA/AMA-M2,组织学表现为胆管系统的损害;5.PSC的临床特点为ALP、GGT及TBIL水平升高,影像学表现为肝内外胆管特征性的病变。
[Abstract]:Objective to analyze the clinical features of autoimmune liver disease and to provide more evidences for the diagnosis and differential diagnosis of autoimmune liver disease. Methods from January 2010 to September 2016, 125 patients with autoimmune liver disease diagnosed by General Hospital of Ningxia Medical University were collected. This study included 38 cases of autoimmune hepatitis and 82 cases of primary biliary cholangitis, including 5 cases of primary sclerosing cholangitis and 5 cases of primary sclerosing cholangitis. The general situation, clinical manifestation, complications, laboratory examination were analyzed and summarized. Imaging and histopathological features. Results the average age of AILD was 58.00 卤13.26 years old, and the ratio of male to female was 1: 4.95. There was no difference in sex composition and age in each group. The main biochemical indexes of AIH group were the elevation of alt AST in PBC group and PSC group. The level of ALB in all three groups was lower than that in PBC group and PSC group. However, the level of IgG in AIH group was higher than that in PBC group and PSC group. The level of IgG in AIH group was higher than that in PBC group P0.05 PBC group, and the level of IgM in AIH group was higher than that in PIH group P0.05 PSC group. There was no significant difference between AIH group and PBC group in the positive rate of ANA, but in AIH group there was no difference in AIH group. The ASMA positive rate of AIH in PBC group was higher than that in PBC group (P 0.05). LKM-1 SLA-P / LP and LC-1 were more specific autoantibodies to AIH. AMA-M2 was PBC specific autoantibody. No specific autoantibodies were found in PSC. There was no difference in the pathological changes of celiac effusion between the patients with AIH and PBC by abdominal color ultrasound and CT, and no cirrhosis and hydroceliac lesions were found in PSC. Abdominal CT showed dilatation of intrahepatic and extrahepatic bile ducts. AIH and PBC showed no bile duct lesions in MRCP / ERCP, while PSC showed withered branches of intrahepatic and extrahepatic bile ducts, and stiff and segmental stricture. AIH showed hepatocyte degeneration. The PBC of necrotic group was small bile duct hyperplasia and cholestasis. Conclusion 1. AILD is more common in middle-aged and elderly women. 2. The clinical features of AILD are that the level of ASTIGG in AILD is higher than that in AIH, and the clinical manifestation of AILD is lack of specificity. The common autoantibodies were Ana Asma, and the specific autoantibodies were LKM-1 / SLAP / LPnLC-1.The histologic features of PBC were liver parenchymal lesions. 4. The clinical features of PBC were the elevation of ALPG-GTTTBIL and IgM levels. The specific autoantibodies were AMA / AMA-M2.The histologic features of PSC were as follows: the lesions of bile duct system. The clinical features of PSC were elevated levels of GGT and TBIL, and the imaging findings were characteristic lesions of intrahepatic and extrahepatic bile ducts.
【学位授予单位】：宁夏医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R575

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