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原发性胆汁性肝硬化与干燥综合征四例分析 预览 被引量:5

Primary biliary cirrhosis and its relation with sj(o)gren syndrome
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摘要 目的探讨原发性胆汁性肝硬化(PBC)与干燥综合征(SS)的关系,提高临床医师对PBC的诊治水平.方法分析4例PBC或SS患者肝损害的临床病理表现、治疗和转归,并复习有关文献.结果 3例PBC患者抗线粒体抗体M2阳性,生化检查及肝穿刺病理检查均提示胆汁淤积性肝损害,其中2例IgM升高.此3例均同时伴有口干燥症和眼干燥症,2例更符合2002年修订的干燥综合征国际分类标准,熊去氧胆酸治疗有效;而第4例单纯干燥综合征患者表现为非胆汁淤积性肝损害,以IgG升高为主,免疫抑制剂治疗有效.结论 PBC肝损害以胆汁淤积为主,抗线粒体抗体M2阳性对PBC诊断有较高特异性,熊去氧胆酸为治疗首选;PBC常伴发SS,二者密切关联又有明显不同. Objective To investigate primary biliary cirrhosis(PBC) and its relation with sjgren syndrome(SS) so as to improve clinical management of PBC.Methods The clinical and pathological manifestations,treatment and outcome of four cases with PBC and/or SS with liver involvement were analyzed and related literature was reviewed. Results 3 cases were positive of antimitochondrial antibody(AMA)and its M2 subtype with predominant cholestatic liver lesion manifested by liver enzyme tests and liver biopsies and responded to ursodeoxycholic acid therapy.All these three cases had xerostomia and keratoconjunctivitis sicca with two fulfilling 2002 international revised classification criteria of sjgren syndrome.Non-cholestatic liver lesion and IgG elevation was seen in the fourth SS case and responded to immunosuppressive treatment.Conclusions PBC manifests with predominant cholestatic liver involvement and often associates and complicates with SS;For PBC,AMA-M2 is a specific antibody and ursodeoxycholic acid should be the first choice for the treatment.
作者 张烜 刘炜 曾小峰 张奉春 董怡 Zhang Xuan,Liu Wei,Zeng Xiaofeng,et al.Department of Rheumatology ,Peking Union Medical College Hospital, Beijing 100730,China
机构地区 北京
出处 《临床内科杂志》 CAS 2005年第1期 31-33,共3页 Journal of Clinical Internal Medicine
关键词 原发性胆汁性肝硬化 干燥综合征 抗线粒体抗体 Primary biliary cirrhosis Sjgren syndrome Antimitochondrial antibody
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