目的　探讨原发性胆汁性肝硬化与干燥综合征的相关性。方法　对 2 0例原发性胆汁性肝硬化伴或不伴发干燥综合征病例的临床特点、血清学检查、肝脏病理、治疗等进行回顾性分析。结果　原发性胆汁性肝硬化病人临床以黄疸、皮肤瘙痒、肝大、脾大为主要表现 ,抗线粒体抗体M2亚型为特征性抗体。合并口眼干燥表现的不少见 ,但不具备原发性干燥综合征的典型表现。治疗上激素和免疫抑制剂疗效不佳 ,熊去氧胆酸有一定的疗效。结论　原发性胆汁性肝硬化合并的干燥综合征不同于原发性干燥综合征 Objective To investigate the relationship between primary biliary cirrhosis and Sjogren’s syndrome. Methods The clinical features, serological tests, liver pathology and treatment of 20 cases of primary biliary cirrhosis with or without Sjogren’s syndrome were retrospectively analyzed. Results Primary biliary cirrhosis patients with jaundice, pruritus, hepatomegaly, splenomegaly as the main performance, anti-mitochondrial antibody M2 subtype characteristic antibodies. Meridian dry mouth performance is not uncommon, but do not have the typical performance of primary Sjogren’s syndrome. Treatment of hormone and immunosuppressive agents ineffective, ursodeoxycholic acid has a certain effect. Conclusions Primary biliary cirrhosis with Sjogren’s syndrome is different from primary Sjogren’s syndrome