原发性胆汁性肝硬化伴自身免疫性肝炎特征的疗效观察

发布时间：2018-10-30 18:19

【摘要】：研究背景自身免疫性肝病(autoimmune liver disease,AILD)是一组因为自身免疫功能紊乱而引起肝细胞或胆管上皮细胞受损的疾病,最常见的有三大类,分别为自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)。此外,一些患者可同时或先后呈现两种或两种以上的自身免疫性肝病的重叠,概括为重叠综合征(overlap syndromes,OS)。其中,最常见的是AIH-PBC OS,其诊断标准及治疗已有了较多的研究。近期我们在临床实践中发现少数PBC的患者虽可伴有AIH特征,但却同时达不到1999年更新的AIH积分系统和巴黎标准的诊断要求,这类患者的诊断介于PBC和AIH-PBC OS之间,称为PBC伴AIH特征(characteristics of primary biliary cirrhosis with autoimmune hepatitis)。其相关诊断及治疗尚无相关报道,是否在熊去氧胆酸(ursodeoxycholic acid,UDCA)的基础上积极加用免疫抑制仍需要探究。故本文总结及分析了31例PBC伴AIH特征患者联合应用UDCA及糖皮质激素(glucocorticoid,GC)的疗效观察结果,以期获得对其更积极的治疗方案。目的研究和探讨熊去氧胆酸联合糖皮质激素治疗PBC伴AIH特征的血清生化学指标、免疫学指标、肝脏病理学表现和免疫抑制治疗效果。方法收集2014年6月~2016年6月期间郑州大学第一附属医院消化内科确诊的PBC伴AIH特征的31例患者,并对其肝脏活检组织进行免疫组化染色。按照治疗方案将统计对象分为单用UDCA组和UDCA联合糖皮质激素组,根据患者肝脏病理炎症(G)和纤维化(S)的不同程度分为两组:炎症程度≤G2、纤维化程度≤S2为病理早期组,炎症程度G2、纤维化程度S2为病理晚期组。收集治疗前后的血清生化指标、免疫相关指标、肝脏组织病理学及应答状态等方面的数据进行总结和分析。结果1.治疗前后两组患者在年龄、性别、临床特点、生化指标(ALT、AST、GGT、ALP、GLOB)、免疫及补体指标(Ig G、Ig M、C3、C4)、肝脏弹性、AIH评分方面的差别无统计学意义(P0.05)。2.单药治疗组GGT、ALP及Ig M较治疗前有所下降,而ALP、Ig M改善有统计学意义(P0.05)。联合治疗组ALT、AST、GGT、ALP、GLOB、Ig G、Ig M、C3及C4较治疗前均有下降,ALT、AST、GLOB、Ig G、C3及C4水平较单药组更低,且具有统计学意义(P0.05)。3.不同治疗组ALT、Ig G、ALP的生化及免疫应答率上无统计学意义(P0.05),但联合治疗组应答率较好。不同病理分期患者ALT应答率有统计学意义(P0.05)。4.联合治疗组患者的肝脏组织炎症程度、纤维化的程度及胆管损害情况较治疗前有明显改善。结论UDCA联合糖皮质激素治疗PBC伴AIH特征的患者,其血清生化、免疫和组织病理指标较单用UDCA组有明显改善。处于病理早期的治疗效果更佳,故建议在UDCA的基础上积极加用免疫抑制治疗,更好的控制疾病的进展。
[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，

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