6例原发性干燥综合征相关肝衰竭临床特征分析  

Clinical characteristics analysis of 6 cases of primary Sjogren’s syndrome related liver failure

作　　者：徐辉[1] 李丽[1] 郑小勤 汪晓军[1] 勾春燕[1] XU Hui;LI Li;ZHENG Xiao-qin;WANG Xiao-jun;GOU Chun-yan(Department of Integrative Traditional Chinese and Western Medicine,Beijing Youan Hospital,Capital Medical University,100069,China)

机构地区：[1]首都医科大学附属北京佑安医院中西医结合科,100069

出　　处：《传染病信息》2022年第6期533-537,共5页Infectious Disease Information

基　　金：2019年度首都医科大学附属北京佑安医院中青年人才孵育项目(BJYAYY-CY2019-12);国家科技重大专项艾滋病和病毒性肝炎等重大传染病防治(2018ZX10725506001013)。

摘　　要：目的分析原发性干燥综合征(primary Sjogren’s syndrome,PSS)相关肝衰竭的临床特点,为该病的临床诊治提供参考。方法收集首都医科大学附属北京佑安医院2010年1月—2020年1月收治的6例PSS相关肝衰竭患者的临床资料,分析其临床特点、诊疗经过和预后。结果6例患者均为女性,平均年龄(67.0±5.5)岁,5例患者在肝衰竭发生前PSS病程为2~16年,1例患者诊断肝衰竭时发现PSS。4例诊断为慢加(亚)急性肝衰竭,2例诊断为慢性肝衰竭。临床以肝衰竭及其并发症症状为主,实验室检查可见肝功能异常,特异性血清学改变为抗核抗体、抗SSA抗体、抗SSB抗体阳性和高球蛋白血症。6例均伴有感染,经积极治疗后,好转率为50%。结论PSS累及肝脏有可能导致肝衰竭,部分患者以慢加急性肝衰竭起病,易并发感染,病死率高。Objective To analyze the clinical characteristics of primary Sjogren’s syndrome(PSS)-related liver failure and to provide reference for the clinical diagnosis and treatment of this disease.Methods The clinical data of 6 patients with PSS-related liver failure admitted to Beijing Youan Hospital affiliated to Capital Medical University from January 2010 to January 2020 were collected,and their clinical characteristics,diagnosis and treatment process and prognosis were analyzed.Results All 6 patients were female,with an average age of(67.0±5.5)years.Five patients had PSS for 2~15 years before the onset of liver failure,and 1 patient was found to have PSS when diagnosed liver failure.Four patients were diagnosed with acute-on-chronic(subacute)liver failure and 2 patients were diagnosed with chronic liver failure.Clinically,liver failure and its complications were the main symptoms,laboratory tests showed abnormal liver function and specific serological changes of anti-nuclear antibody,anti-SSA antibody,anti-SSB antibody positive and hyperglobulinemia.All 6 patients were accompanied with infection,and the improvement rate was 50% after aggressive treatment.Conclusions PSS involves the liver and may lead to liver failure,with some patients starting with acute-on-chronic liver failure,which is prone to complicated infection and high mortality rate.

关 键 词：干燥综合征 肝衰竭 临床特点 预后 

分 类 号：R442.8[医药卫生—诊断学]