本文選題：自身免疫性肝病 + 自身免疫性肝炎��； 參考：《寧夏醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的 分析自身免疫性肝病患者的臨床特點(diǎn),為自身免疫性肝病的診斷和鑒別診斷提供更多依據(jù)。方法 收集2010年1月至2016年9月寧夏醫(yī)科大學(xué)總醫(yī)院確診自身免疫性肝病(AILD)的患者共125例,包括38例自身免疫性肝炎(AIH)、82例原發(fā)性膽汁性膽管炎(PBC)、5例原發(fā)性硬化性膽管炎(PSC),分析總結(jié)患者的一般情況、臨床表現(xiàn)、合并癥情況、實(shí)驗(yàn)室檢查、影像學(xué)及組織病理學(xué)檢查的特點(diǎn)。結(jié)果 AILD好發(fā)于中老年女性,平均年齡為(58.00±13.26)歲,男女比例為1:4.95。各組在性別構(gòu)成及年齡上無差異。各組臨床表現(xiàn)無特異性。AIH組生化指標(biāo)以ALT、AST升高為主,PBC組及PSC組以ALP、GGT、TBIL升高為主,三組ALB水平均降低,但AIH組GLB水平高于PBC組及PSC組(P0.05)。AIH組Ig G水平高于PBC組(P0.05),PBC組Ig M水平高于AIH組(P0.05),PSC組Ig G和Ig M水平無明顯變化。AIH組ANA陽性率與PBC組比較無差別(P0.05),但在ANA分型上,AIH組以斑點(diǎn)型(42.42%)為主,PBC組以胞漿顆粒型(50.75%)為主;AIH的ASMA陽性率高于PBC組(P0.05)。LKM-1、SLA/LP及LC-1是AIH比較特異性的自身抗體,AMA/AMA-M2是PBC特異性的自身抗體,而PSC未發(fā)現(xiàn)特異性的自身抗體。腹部彩超、腹部CT在檢測AIH和PBC患者肝硬化、腹腔積液的病變上比較無差異(P0.05),在PSC中未發(fā)現(xiàn)肝硬化及腹腔積液病變,腹部CT表現(xiàn)為肝內(nèi)外膽管的擴(kuò)張。AIH和PBC在MRCP/ERCP中未發(fā)現(xiàn)膽管病變,而PSC表現(xiàn)為肝內(nèi)外膽管枯樹枝樣改變、僵硬和節(jié)段性狹窄。AIH組織學(xué)表現(xiàn)為肝細(xì)胞變性、壞死,PBC為小膽管增生及膽汁淤積改變。結(jié)論 1.AILD好發(fā)于中老年女性;2.AILD在臨床表現(xiàn)上缺乏特異性;3.AIH的臨床特點(diǎn)為ALT、AST、Ig G水平升高,常見的自身抗體有ANA、ASMA,特異性的自身抗體有LKM-1、SLA/LP、LC-1,組織學(xué)表現(xiàn)為肝臟實(shí)質(zhì)損害;4.PBC的臨床特點(diǎn)為ALP、GGT、TBIL和Ig M水平升高,特異性的自身抗體為AMA/AMA-M2,組織學(xué)表現(xiàn)為膽管系統(tǒng)的損害;5.PSC的臨床特點(diǎn)為ALP、GGT及TBIL水平升高,影像學(xué)表現(xiàn)為肝內(nèi)外膽管特征性的病變。
[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 鹵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.
【學(xué)位授予單位】：寧夏醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R575

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