难治型原发性胆汁性胆管炎患者临床特点分析  

Clinical features of patients with refractory primary biliary cholangitis

作　　者：刘会敏 张洪文[1] 林睿[1] 宋岩 周璐[1] 王邦茂[1] 刘文天[1] Hui-Min Liu;Hong-Wen Zhang;Rui Lin;Yan Song;Lu Zhou;Bang-Mao Wang;Wen-Tian Liu(Department of Gastroenterology,General Hospital of Tianjin Medical University,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院消化内科,天津市300052

出　　处：《世界华人消化杂志》2019年第4期238-244,共7页World Chinese Journal of Digestology

基　　金：天津市科委慢性病防治科技重大专项项目;No.17ZXMFSY00210;天津市卫计委卫生行业重点攻关项目;No.15KG149;国家自然科学基金资助项目;No.81600509

摘　　要：目的分析难治型原发性胆汁性胆管炎(primarybiliarycholangitisPBC)患者的临床特征,探讨影响熊去氧胆酸(ursodeoxycholic acid, UDCA)治疗效果的相关因素,为制定合理的治疗方案提供依据.方法回顾性分析53例经UDCA治疗后的PBC患者,其中15例(28.30%)应答不良,为难治组, 38例(71.70%)获得良好应答,为典型组,比较分析两组患者的临床特点、生化指标、自身抗体、腹部超声检查、肝脏组织学特征及治疗应答反应.结果难治组与典型组均以女性为主,两组男女比例、平均发病年龄及伴肝外自身免疫性疾病等一般状况无明显差异.治疗前难治组谷丙转氨酶(alanine aminotransferase, ALT)、谷草转氨酶(aspartate aminotransferase, AST)、碱性磷酸酶(alkalinephosphatase, ALP)、谷氨酰转肽酶(glutamyltransferase,GGT)、总胆红素(totalbilirubin,TBIL)与直接胆红素(directbilirubin,DBIL)及抗线粒体抗体(anti-mitochondrial antibodies, AMA)均明显高于典型组,差异具有统计学意义(P<0.05).难治组与典型组肝硬化比例分别为46.7%和18.4%;脾大的发病率分别为60%和26.3%;肝内胆汁淤积的比例分别为66.7%和5.3%,差异均具有统计学意义(P <0.05).二元Logistics回归分析发现ALP和肝内胆汁淤积为影响UDCA初始治疗疗效的独立危险因素.结论初治时ALT、AST、ALP、GGT、TBIL、DBIL、AMA水平较高,伴有肝硬化、脾大并有肝内胆汁淤积的组织特征可能是PBC患者对UDCA应答不良的重要影响因素.AIM To analyze the clinical characteristics of refractory primary biliary cholangitis(PBC)and explore the factors affecting the therapeutic effect of ursodeoxycholic acid(UDCA),so as to provide new ideas for the clinical diagnosis and treatment of PBC.METHODS A retrospective analysis was performed on 53 patients with PBC following UDCA treatment.Fifteen patients(28.30%)who had a poor response were included in a refractory group,and 38 patients(71.70%)who had a complete response were included in a typical group.Clinical characteristics,biochemical parameters,autoantibodies,abdominal ultrasonography,liver pathology,and response to treatment were analyzed and compared between the two groups.RESULTS There was a female predominance in both groups.There was no significant difference between the two groups in the gender distribution,the average age of onset,or general conditions such as comcomitant extrahepatic autoimmune diseases.The levels of alanine aminotransferase(ALT),aspartate aminotransferase(AST),alkaline phosphatase(ALP),glutamyltransferase(GGT),total bilirubin(TBIL),direct bilirubin(DBIL),and anti-mitochondrial antibodies(AMA)in the refractory group were significantly higher than those in the typical group before treatment(P<0.05).The proportion of patients with intrahepatic cirrhosis was 46.7%in the refractory group and 18.4%in the typical group;the proportion of patients with splenomegaly was 60%and 26.3%,respectively;the proportion of patients with intrahepatic cholestasis was 66.7%and 5.3%,respectively;the difference between the two groups was statistically significant(P<0.05).Logistic regression analysis demonstrated that ALP and cholestasis were independent risk factors affecting the efficacy of initial treatment of UDCA.CONCLUSION High levels of ALT,AST,ALP,GGT,TBIL,DBIL and AMA at the initial treatment as well as concomitant cirrhosis,splenomegaly,and intrahepatic cholestasis may be closely related to a poor response to UDCA-based treatment.

关 键 词：原发性胆汁性胆管炎 熊去氧胆酸 自身免疫性疾病 难治型 

分 类 号：R575.22[医药卫生—消化系统]