原发性胆汁淤积性肝硬化合并系统性红斑狼疮分析

目的探讨原发性胆汁性肝硬化（PBC）合并系统性红斑狼疮（SLE）的发生情况及治疗效果。方法回顾性分析2008年1月至2014年12月PBC患者1380例中合并SLE的临床特点。结果PBC合并SLE者共58例，占全部PBC患者的4．2％，女性患者占93．10％。40例首先出现SLE，184a4首先出现PBC。PBC合并SLE组（A组）首发症状皮肤瘙痒、口眼干发生率明显低于PBC组（B组），但发热高于后者（P〈0．05）；A组白细胞计数、血小板计数、血红蛋白、碱性磷酸酶、γ-谷氨酰转肽酶水平低于B组（P〈0．05），血清免疫球蛋白G（IgG）水平明显高于B组（P〈0．05）。A组患者皆用熊去氧胆酸＋强的松＋免疫抑制剂联合治疗，短期内显示一定疗效。结论PBC合并SLE患者并不少见，首发症状以发热多见，口眼干、皮肤瘙痒少见，更易合并血三系降低及血清IgG升高，但PBC的肝损害相对较轻。SLE可能是PBC的一个保护性因素。

Objective We aimed to investigate the characteristics of primary biliary cirrhosis （ PBC ） developed in patients with systemic lupus erythematosus （ SLE ） . Methods A retrospective analysis of 58 cases of PBC patients with SLE were reviewed. Results 4.2% PBC patients overlapped with SLE. Among 58 patients 54 were female, and their age at the diagnosis were （ 34.82 ± 10.28 ） years old. The incidence of fever were significantly higer in PBC with SLE patients （ P〈0.05 ） , but the incidence of dryeye drymonth and pruritus were lower （ P〈0.05 ） . PBC with SLE patients had lower WBC, Hb, PLT counts and lower serum level of ALP, γ -GGT than PBC patients. All the PBC with SLE patients were subjected to combined treatment of ursodeoxycholic acid and corticosteroid and immunosupresive agents. Conclusion The coexistence of PBC and SLE is not rare. SLE may be one of the protective factors of PBC.