中文摘要：

目的随诊和总结熊去氧胆酸（UDCA）治疗1年以上的难治性原发性胆汁性肝硬化（PBC）患者与典型PBC患者的免疫学特点异同。方法60例随诊1年以上的PBC患者，根据对UDCA应答情况分为应答良好组（典型组，37例）和应答不良组（难治性组，23例），以年龄、性别匹配的健康志愿者20名（健康对照组），检测各组外周血淋巴细胞亚群、细胞因子等免疫学特点。统计学分析采用独立样本t检验或单因素方差分析。结果①在治疗超过1年的稳定期时，难治性和典型PBC外周血淋巴细胞亚群的差异无统计学意义，但2组的B细胞、T细胞、CD4＋T细胞、CD4＋CD28＋T细胞和CD8‘CD28一T细胞比例均高于健康对照组；②在治疗超过1年的稳定期时，难治性组血清白细胞介素（IL）一6水平[（0．8＋0．9）pg／m1]高于典型组[（0．3＋0．4）pg／m1]；血清肝细胞生长因子（HGF）水平呈现难治性组〉典型组〉健康对照组趋势。结论经过1年以上治疗后，对UDCA应答不良的患者的血清IL．6、HGF等细胞因子较高，提示可能存在较应答良好患者更重的免疫紊乱。

英文摘要：

Objective To describe the immunological characteristics of refractory primary biliary cirrhosis compared with the typical patients for more than 1 year＇s administration of UDCA. Methods Sixty patients treated with UDCA for more than 1 year in our clinic were enrolled into this study. According to the response to UDCA by Paris criteria, patients were divided into refractory group （23 patients） and typical groups （37 patients）. The recent peripheral lymphocyte subsets and cytokines of the two groups were tested and analyzed. One-way ANOVA and t test were used for statistical analysis. Results （~ One-year treatment after diagnosis, there were no differences between the two groups in the distribution of peripheral lymphocytic subsets, meanwhile, the two groups had higher percentage of B cells, CD4~ T cells, CD4＋ CD28~ T cells and CD8~CD28-T cells than healthy controls respectively. （~） The serum levels of IL-6 [ （0.8_＋0.9） pg/ml vs （0.3_＋ 0.4） pg/ml ] and HGF were higher in the refractory group than other groups. Conclusion During the plateau phase, refractory PBC patients have higher serum levels of IL-6 and HGF, which probably suggest that the refractory PBC patients may have severe immunologic disturbance in vivo.