| 119 | 1 | 91 |
| 下载次数 | 被引频次 | 阅读次数 |
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。
Abstract：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.
[1] Kaplan MJ, Ike RW. The liver is a common non-exocrine target in primary Sjogren’ s syndrome:a retrospective review[J]. BMC Gastroenterol, 2002, 2:21. DOI:10.1186/1471-230X-2-21.
[2]张文，厉小梅，徐东，等.原发性干燥综合征诊疗规范[J].中华内科杂志，2020,59(4):269-276. DOI:0.3760/cma.j.cn112138-20200113-00021.
[3]中华医学会感染病学分会肝衰竭与人工肝学组，中华医学会肝病学分会重型肝病与人工肝学组.肝衰竭诊治指南（2018年版）[J].国际流行病学传染病学杂志，2018,45(6):379-387. DOI:10.3969/j.issn.2096-3351.2019.02.001.
[4]中华医学会肝病学分会.原发性胆汁性胆管炎的诊断和治疗指南（2021）[J].临床肝胆病杂志，2022,38(1):35-41.DOI:10.3760/cma.j.cn112138-20211112-00794-1.
[5]中华医学会肝病学分会.自身免疫性肝炎诊断和治疗指南（2021）[J].临床肝胆病杂志，2022,38(1):42-49. DOI:10.3760/cma.j.cn112138-20211112-00796-1.
[6]中华医学会肝病学分会.原发性硬化性胆管炎诊断及治疗指南（2021）[J].临床肝胆病杂志，2022,38(1):50-61.DOI:10.3760/cma.j.cn112138-20211109-00786-1.
[7]梁连春，孟庆华，刘德恭.干燥综合征致肝损害误诊为肝炎[J].临床误诊误治，2002,15(3):196-197. DOI:10.3969/j.issn.1002-3429.2002.03.035.
[8]李丽，勾春燕，许文君，等.成人斯蒂尔病伴严重肝功能异常临床分析7例[J].世界华人消化杂志，2016,24(3):431-435. DOI:10.11569/wcjd.v24.i3.431.
[9]郑寒晨.非肝源性自身免疫性肝损伤患者的临床特点回顾性分析[D].济南：山东大学，2020.
[10] Podgorska J, Werel P, Klapaczynski J, et al. Liver involvement in rheumatic diseases[J]. Reumatologia, 2020, 58(5):289-296. DOI:10.1016/0049-0172(82)90027-0.
[11]中华医学会风湿病学分会.干燥综合征诊断及治疗指南[J].中华风湿病学杂志，2010,14(11):766-768. DOI:10.3760/cma.j.issn.1007-7480.2010.11.011.
[12] Valim V, Zandonade E, Pereira AM, et al. Primary Sjogren’ s syndrome prevalence in a major metropolitan area in Brazil[J].Rev Bras Reumatol, 2013, 53(1):24-34. DOI:10.1016/S 2255-5021(13)70003-8.
[13] Fauchais AL, Martel C, Vidal E.[Epidemiology and physiopathogy of Sjogren’ s syndrome][J]. Rev Prat, 2012,62(2):218-220. DOI:10.1016/j.semarthrit.2005.07.001.
[14]何菁，丁艳，李玉慧，等.原发性干燥综合征患者初诊的临床特征分析[J].北京大学学报（医学版），2012,44(2):225-228. DOI:10.3969/j.issn.1671-167X.2012.02.013.
[15]张卓莉，董怡.原发性干燥综合征肝脏损害的临床及免疫学特点（附30例临床分析）[J].中华风湿病学杂志，1998,2(2). DOI:10.3760/j:issn:1007-7480.1998.02.009.
[16]梁贺梅，关劼，祖宁.原发性干燥综合征肝损害临床分析[J].华北煤炭医学院学报，2002,4(5):570-571. DOI:10.3969/j.issn.1008-6633.2002.05.014.
[17]刘钢，程小云，刘怡欣.原发性干燥综合征导致肝脏损害的32例临床分析[J].免疫学杂志，2004,20(3):227-230.DOI:10.3969/j.issn.1000-8861.2004.03.017.
[18] Skopouli FN, Barbatis C, Moutsopoulos HM. Liver involvement in primary Sjogren’ s syndrome[J]. Br J Rheumatol, 1994,33(8):745-748. DOI:10.1093/rheumatology/33.8.745.
[19] Montano-Loza AJ, Crispin-Acuna JC, Remes-Troche JM, et al.Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjogren’ s syndrome[J]. Ann Hepatol,2007, 6(3):150-155. DOI:10.1111/j.1572-0241.2006.00947.x.
[20] Lindgren S, Manthorpe R, Eriksson S. Autoimmune liver disease in patients with primary Sjogren’ s syndrome[J].J Hepatol, 1994, 20(3):354-358. DOI:10.1016/S0168-8278(94)80007-3.
[21]袁小燕.干燥综合征合并自身免疫性肝炎的临床分析[J].江苏医药, 2005, 31(4):310-310. DOI:10.3969/j.issn.0253-3685.2005.04.031.
[22]陈伟钱，戴小娜，余叶，等.原发性干燥综合征合并自身免疫性肝病的临床特点及预后分析[J].北京大学学报（医学版），2020,52(5):886-891. DOI:10.19723/j.issn.1671-167X.2020.05.015.
[23]何菁，李春，强璐，等.原发性干燥综合征合并肝损伤的临床特点及独立危险因素分析[J].中华全科医师杂志，2010,9(4):248-251. DOI:10.3760/cma.j.issn.1671-7368.2010.04.010.
[24]侯环荣，尚佳，康谊，等.以急性肝衰竭为首发症状的自身免疫性肝炎的临床分析[J].胃肠病学和肝病学杂志，2016,25(4):426-427. DOI:10.3969/j.Issn.1006-5709.2016.04.017.
[25] Moutsopoulos HM. Sjogren’ s syndrome or autoimmune epithelitis?[J]. Clin Ren Allergy Immunol, 2007, 32(3):199-200. DOI:10.1007/s12016-007-8042-1.
[26]朱林林，杨李桦，谢其冰，等.自身免疫性肝炎合并干燥综合征的临床病理特点分析[J].四川大学学报（医学版），2018,49(2):183-187. DOI:10.13464/j.scuxbyxb.2018.02.005.
[27]陶弘.自身免疫性肝病合并干燥综合征的临床分析[D].大连：大连医科大学，2021.
[28]中中华医学会肝病学分会药物性肝病学组.药物性肝损伤诊治指南[J].实用肝脏病杂志，2017,20(2):后插1-后插18. DOI:10.3969/j.issn.1672-5069.2017.02.039.
[29]中华医学会肝病学分会.肝硬化诊治指南[J].中华肝脏病杂志，2019,27(11):846-865. DOI:10.3969/j.issn.1001-5256.2019.11.006.
[30] Youssef WI, Tavill AS. Connective tissue diseases and the liver[J]. J Clin Gastroenterol, 2002, 35(4):345-349. DOI:10.1097/00004836-200210000-00012.
[31] Czaja AJ. Autoimmune hepatitis. Evolving concepts and treatment strategies[J]. Dig Dis Sci, 1995, 40(2):435-456. DOI:10.1007/BF02065434.
[32]伍沪生，宋慧，黄彦弘，等.原发性干燥综合征的肝脏损害[J].中华风湿自身免疫性肝炎合并干燥综合征的临床病理特点分析病学杂志，2001,5(1):29-31. DOI:10.3760/j:issn.1007-7480.2001.01.008.
[33]林磊.原发性干燥综合征肝脏损害临床特点及转归[J].饮食保健，2018,5(12):103-104. DOI:10.3969/j.issn.2095-8439.2018.12.125.
[34]孙朝霞，张洁利，倪秀莹等.肝硬化并发肝衰竭患者发生血流感染的预后因素分析[J].传染病信息，2020,33(3):225-228. DOI:10.3969/j.issn.1007-8134.2020.03.008.
[35]朱冰，游绍莉，胡瑾华，等.《2019年APASL慢加急性肝衰竭共识建议（更新）》要点[J].传染病信息，2019,32(4):289-298. DOI:10.3969/j.issn.1007-8134.2019.04.001.
基本信息:
中图分类号:R593.2;R575.3
引用信息:
[1]徐辉,李丽,郑小勤,等.6例原发性干燥综合征相关肝衰竭临床特征分析[J].传染病信息,2022,35(06):533-537.
基金信息:
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）
2022-12-30
2022-12-30