原发性胆汁性肝硬化与干燥综合征20例临床分析

Clinical characteristics of primary biliary cirrhosis with Sjgren′s syndrome of 20 cases

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。

Objective Primary biliary cirrhosis (PBC) is a chronic non suppurative destructive cholangitis of unknown etiology. It has been reported that patients with PBC suffer from xerostomia and keratoconjunctivitis at high prevalence and PBC has been shown to be associated with Sjgren′s syndrome. This study investigated the relationship between PBC and Sjgren′s syndrome. Method 20 cases diagnosed as PBC consented to the evaluation for Sjgren′s syndrome which included clinical and serological features, Schirmer′s test, measurement of parotid flow rate and labial minor salivary gland biopsy. Results The main manifestations of PBC are jaundice, pruritus, hepatosplenomegaly. M2 subtype of antimitochondrial antibody is of great specificity for PBC. Many PBC patients are suffering from xerostomia and keratoconjunctivitis sicca but do not have typical serological and pathological presentations of primary Sjgren′s syndrome. Corticosteroids and immunosuppressive agents have disappointing effects in the treatment. Ursodeoxycholic acid is effective to certain extent. Conclusion It was indicated that Sjgren′s syndrome associated with PBC appears to be a secondary form differing from primary Sjgren′s syndrome.