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目的探讨胆汁淤积在原发性干燥综合征(pSS)中的意义及其与原发性胆汁性肝硬化(PBC)的关联。方法将81例肯定的pSS患者按抗线粒体抗体(AMA)阳性与否分为两组,前瞻性地追随观察其临床表现、病程、实验室检查、转归有无异同。胆汁淤积标准定为血总胆红素(TBil)、碱性磷酸酶(ALP)、酌-谷氨酰转移酶(酌-GT)至少两项较正常增高1倍以上。结果AMA(+)组(46例)与AMA(-)组(35例)比较,在性别、年龄、血TBil、ALP、酌-GT,单项IgG、单项IgM增高及血抗核抗体(ANA)、类风湿因子(RF)、抗SSA和/或SSB抗体,抗着丝点抗体阳性例数上差异均无显著性。两组患者一般情况较好,皆有肝功能异常史长达20年的病例。两组皆有患者有干燥综合征(SS)症状或确诊为SS已多年,而只近年才出现胆汁淤积。20例AMA(+)及14例AMA(-)者查血核周型抗中性粒细胞胞质抗体(pANCA),前组1例阳性,后组3例阳性,其中2例行逆行胆管造影,皆示有硬化性胆管炎。9例AMA(+)及10例AMA(-)者行肝活检,除1例未见足够汇管区难作结论外,余均示汇管区及胆管炎症存在。81例皆予熊去氧胆酸、泼尼松、免疫抑制剂联合治疗,胆汁淤积很难完全消退,但前瞻性追随观察(最长者6年),绝大多数病例情况维持良好。结论①pSS所致胆管炎引起胆汁淤积可能并不少见,宜称之为pSS胆管炎而不宜诊为
Objective To investigate the significance of cholestasis in primary Sjogren’s syndrome (pSS) and its relationship with primary biliary cirrhosis (PBC). Methods Eighty-one positive patients with pSS were divided into two groups according to their positive anti-mitochondrial antibodies (AMA), and their clinical manifestations, course of disease and laboratory tests were followed up prospectively. Cholestasis criteria for the determination of blood total bilirubin (TBil), alkaline phosphatase (ALP), discretion - glutamyl transferase (discretion-GT) at least two more than two times the normal increase. Results AMA (+) group (46 cases) and AMA (-) group (35 cases) were significantly higher than those in AMA group (35 cases) , Rheumatoid factor (RF), anti-SSA and / or SSB antibodies, anti-centromere antibody positive cases no significant difference. The two groups of patients generally good, all have history of abnormal liver function up to 20 years of cases. Patients in both groups had symptoms of SS or had been diagnosed with SS for many years, whereas cholestasis occurred only in recent years. Peripheral blood anti-neutrophil cytoplasmic antibody (pANCA) was detected in 20 patients with AMA (+) and 14 patients with AMA (-). One patient was positive in the former group and 3 patients were positive in the latter group. Two of them were retrograde cholangiography , All showed sclerosing cholangitis. Nine patients with AMA (+) and 10 patients with AMA (-) underwent liver biopsy. Except for 1 case that there was no sufficient portal area, the results of ECT and bile duct inflammation were all shown. All of the 81 cases were treated with ursodeoxycholic acid, prednisone and immunosuppressive agents. Cholestasis was difficult to completely disappear. However, the patients were followed up prospectively (the longest was 6 years). The majority of cases were well maintained. Conclusion ① cholangitis caused by cholangiitis cholestasis may not be uncommon, it should be called pSS cholangitis should not be diagnosed as