原发性干燥综合征的肝脏损害

Liver damage in primary Sjgren′s syndrome

目的 研究原发性干燥综合征 (pSS)合并肝脏损害的临床特点 ,探讨其病理本质。方法 分析 5 6例 pSS病例中合并肝脏损害及不伴肝脏损害的临床资料。 结果 5 6例 pSS病例中 13例合并肝脏损害 ,发生率为 2 3 2 % ,其性别、年龄、从发病到确诊时间以及ANA、抗SSA、抗SSB、类风湿因子、IgG、γ 球蛋白阳性率方面与不伴肝脏损害之间的差异均无显著性 (P >0 0 5 )。 13例中 6例AKP、γ GT同时升高 ,4例AKP、γ GT、TBIL、DBIL同时升高。查抗平滑肌抗体 (抗SMA)、抗线粒体抗体 (AMA)的 8例中 ,5例AMA阳性。 6例进行肝穿刺活组织检查 ,病理诊断为慢性活动性肝炎 3例 ,其中 2例合并肝硬化 ;慢性迁延性肝炎 1例 ,小胆管炎 2例。 6例中 5例均有不同程度的汇管区单个核细胞浸润。结论 pSS合并肝脏损害并不少见 ,可能与原发性胆汁性肝硬化相关。患者对激素治疗敏感 ,预后较好。

Objective To study clinical features of the liver damage in primary Sjgren′s syndrome (pSS) and to investigate the nature of their pathological changes.Method Clinical data of 56 pSS patients with liver damage and without liver damage were analyzed.Results Among 56 patients with pSS,13 (23 2%) showed liver damage.The positive rates of sex,age,duration from symptom appearence to definite diagnosis,ANA,anti SSA,anti SSB,RF,IgG and γ globulin revealed no significant difference between both groups ( P >0 05).Among the 13 patients with liver damage,AKP and γ GT were raised in 6,and AKP,γ GT,TBIL and DBIL all elevated in 4 In 8 patients anti SMA and AMA were detected,and 5 showed AMA positive.Liver biopsy in 6 patients showed 3 with chronic active hepatitis among which 2 were complicated with liver cirrhosis,1 chronic persistent hepatitis and 2 cholangitis.Of the 6 patients 5 showed different degrees of infiltration of mononuclear cells in the portal tracts.Conclusion The occurrence of liver damage in pSS is rather high.The liver damage may be related to primary biliary cirrhosis (PBC).Patients′ response to corticosteroid treatment is favourable and their prognosis appears good.