【摘要】：研究背景自身免疫性肝病(autoimmune liver disease,AILD)是一組因為自身免疫功能紊亂而引起肝細胞或膽管上皮細胞受損的疾病,最常見的有三大類,分別為自身免疫性肝炎(autoimmune hepatitis,AIH)、原發(fā)性膽汁性肝硬化(primary biliary cirrhosis,PBC)、原發(fā)性硬化性膽管炎(primary sclerosing cholangitis,PSC)。此外,一些患者可同時或先后呈現(xiàn)兩種或兩種以上的自身免疫性肝病的重疊,概括為重疊綜合征(overlap syndromes,OS)。其中,最常見的是AIH-PBC OS,其診斷標準及治療已有了較多的研究。近期我們在臨床實踐中發(fā)現(xiàn)少數(shù)PBC的患者雖可伴有AIH特征,但卻同時達不到1999年更新的AIH積分系統(tǒng)和巴黎標準的診斷要求,這類患者的診斷介于PBC和AIH-PBC OS之間,稱為PBC伴AIH特征(characteristics of primary biliary cirrhosis with autoimmune hepatitis)。其相關診斷及治療尚無相關報道,是否在熊去氧膽酸(ursodeoxycholic acid,UDCA)的基礎上積極加用免疫抑制仍需要探究。故本文總結(jié)及分析了31例PBC伴AIH特征患者聯(lián)合應用UDCA及糖皮質(zhì)激素(glucocorticoid,GC)的療效觀察結(jié)果,以期獲得對其更積極的治療方案。目的研究和探討熊去氧膽酸聯(lián)合糖皮質(zhì)激素治療PBC伴AIH特征的血清生化學指標、免疫學指標、肝臟病理學表現(xiàn)和免疫抑制治療效果。方法收集2014年6月~2016年6月期間鄭州大學第一附屬醫(yī)院消化內(nèi)科確診的PBC伴AIH特征的31例患者,并對其肝臟活檢組織進行免疫組化染色。按照治療方案將統(tǒng)計對象分為單用UDCA組和UDCA聯(lián)合糖皮質(zhì)激素組,根據(jù)患者肝臟病理炎癥(G)和纖維化(S)的不同程度分為兩組:炎癥程度≤G2、纖維化程度≤S2為病理早期組,炎癥程度G2、纖維化程度S2為病理晚期組。收集治療前后的血清生化指標、免疫相關指標、肝臟組織病理學及應答狀態(tài)等方面的數(shù)據(jù)進行總結(jié)和分析。結(jié)果1.治療前后兩組患者在年齡、性別、臨床特點、生化指標(ALT、AST、GGT、ALP、GLOB)、免疫及補體指標(Ig G、Ig M、C3、C4)、肝臟彈性、AIH評分方面的差別無統(tǒng)計學意義(P0.05)。2.單藥治療組GGT、ALP及Ig M較治療前有所下降,而ALP、Ig M改善有統(tǒng)計學意義(P0.05)。聯(lián)合治療組ALT、AST、GGT、ALP、GLOB、Ig G、Ig M、C3及C4較治療前均有下降,ALT、AST、GLOB、Ig G、C3及C4水平較單藥組更低,且具有統(tǒng)計學意義(P0.05)。3.不同治療組ALT、Ig G、ALP的生化及免疫應答率上無統(tǒng)計學意義(P0.05),但聯(lián)合治療組應答率較好。不同病理分期患者ALT應答率有統(tǒng)計學意義(P0.05)。4.聯(lián)合治療組患者的肝臟組織炎癥程度、纖維化的程度及膽管損害情況較治療前有明顯改善。結(jié)論UDCA聯(lián)合糖皮質(zhì)激素治療PBC伴AIH特征的患者,其血清生化、免疫和組織病理指標較單用UDCA組有明顯改善。處于病理早期的治療效果更佳,故建議在UDCA的基礎上積極加用免疫抑制治療,更好的控制疾病的進展。
[Abstract]:Background autoimmune liver disease (autoimmune liver disease,AILD) is a group of diseases that cause damage to hepatocytes or bile duct epithelial cells due to autoimmune dysfunction. The most common diseases are autoimmune hepatitis (autoimmune hepatitis,AIH). Primary biliary cirrhosis (primary biliary cirrhosis,PBC), primary sclerosing cholangitis (primary sclerosing cholangitis,PSC). In addition, some patients may present overlap of two or more autoimmune liver diseases at the same time or in succession, which can be summed up as overlap syndrome (overlap syndromes,OS). Among them, the most common is AIH-PBC OS, its diagnostic criteria and treatment has been a lot of research. In recent clinical practice, we found that a small number of patients with PBC may be accompanied by AIH features, but at the same time they do not meet the diagnostic requirements of the AIH integral system and the Paris standard, which were updated in 1999. The diagnosis of these patients is between PBC and AIH-PBC OS. Called PBC with AIH feature (characteristics of primary biliary cirrhosis with autoimmune hepatitis). The diagnosis and treatment of ursodeoxycholic acid (ursodeoxycholic acid,UDCA) have not been reported. Whether to add immunosuppression to ursodeoxycholic acid (ursodeoxycholic acid,UDCA) still needs to be explored. Therefore, 31 patients with PBC and AIH were treated with UDCA and glucocorticoid (glucocorticoid,GC) in order to obtain a more active treatment. Objective to study the effects of ursodeoxycholic acid combined with glucocorticoid in the treatment of PBC with AIH. Methods from June 2014 to June 2016, 31 patients with PBC with AIH characteristics confirmed in the Department of Digestive Medicine, the first affiliated Hospital of Zhengzhou University, were collected and their liver biopsy tissues were stained with immunohistochemistry. According to the treatment plan, the statistical subjects were divided into UDCA group and UDCA combined with glucocorticoid group. According to the degree of pathological inflammation (G) and fibrosis (S) in patients, they were divided into two groups: inflammation degree 鈮，

本文編號：2300791