结缔组织病并发早期原发性胆汁性肝硬化临床分析  

Connective tissue diseases complicated with early primary biliary cirrhosis

作　　者：池淑红[1] 朱彩霞[1] 李海波[1] 鱼云霞[1] 

机构地区：[1]宁夏医科大学附属医院,宁夏银川750004

出　　处：《宁夏医学杂志》2011年第8期713-715,共3页Ningxia Medical Journal

摘　　要：目的分析结缔组织病(CTD)并发早期原发性胆汁性肝硬化(PBC)的临床特点(类型、肝脏生化指标、免疫学指标)、治疗和转归。方法对11例CTD并发早期PBC的临床资料进行回顾性分析。结果病例CTD以干燥综合征(SS)居多,其次为系统性红斑狼疮(SLE)、类风湿关节炎(RA)、未分化结缔组织病(UCTD)、多发性肌炎(PM),病程最长8年,最短4个月。多数无PBC症状,仅2例有黄疸、皮肤瘙痒、乏力表现,肝脏生化指标均以胆酶升高为主,AMA和(或)AMA-M2阳性;ANA荧光核型多样,以胞浆颗粒型为主,免疫球蛋白IgM升高较明显;经糖皮质激素GC、免疫抑制剂和熊去氧胆酸(ODCA)治疗,随访的10例患者中,8例病情有改善。结论 CTD并发PBC并不少见,涉及的疾病谱较广,早期症状不典型,胆酶的升高及AMA、AMA-M2阳性是PBC早期诊断的主要依据,ANA的荧光模型、IgM的增高亦有重要的临床意义,糖皮质激素、免疫抑制剂和熊去氧胆酸治疗有效。Objective To discuss the clinical features（types,biochemical indexes of liver,immunological indexes）,treatment and outcome of connective tissue diseases（CTD） complicated with early primary biliary cirrhosis（PBC）.Methods 11 cases with CTD complicated with early PBC were retrospectively analyzed.Results Among all the cases,Sjogren＇s syndrome was most common,followed by systemic lupus erythematosus,rheumatoid arthritis,undifferentiated connective tissue disease and multiple muscle inflammation.The course of disease lasted for 4 months to 8 years.Most of the patients had no PBC symptoms;only 2 cases of them had jaundice,itchy of skin and fatigue.The biochemical indexes of liver showed elevated alkaline phosphatase（AKP） and γ-glutamyltransferase（GGT）.Immunological indexes showed positive AMA and AMA-M2,obviously elevated IgM,diversity in ANA fluorescence karyotype and mainly cytoplasm particle type.After the treatment of glucocorticoid,immunosuppressants and ursodeoxycholic acid,among the 10 patients who were followed up,8 patients＇ condition were improved.Conclusion CTD complicated with PBC is not uncommon,involving wide spectrum of disease and the early manifestations are not typical.Elevated AKP and GGT,positive AMA and AMA-M2 are main stay of early diagnosis of PBC.The ANA fluorescence karyotype and elevated IgM also have important clinical significance.Treatment of glucocorticoid,immunosuppressants and Ursodeoxycholic acid is effective.

关 键 词：原发性胆汁性肝硬化 结缔组织病 早期诊断 

分 类 号：R575.2[医药卫生—消化系统]