自身免疫性肝炎和原发性胆汁性肝硬化重叠综合征的临床特征及疗效观察

摘要

Objective To observe the clinical feature and curative efficacy of AIH - PBC overlap syndrome. Methods Study 1: Clinical features of 124 patients with primary biliary cirrhosis, 57 patients with autoimmune hepatitis and 39 patients with AIH - PBC overlap syndrome were retrospectively analyzed. Study 2: Curative efficacy of 39 patients with AIH - PBC overlap syndrome were grouping analyzed based on different treatment schedule. Results AIH - PBC overlap syndrome constituted 17.73% of 220 patients with autoimmune liver diseases. No significant differences were found in sex ratio and symptoms of fatigue and jaundice among three groups. But there were significance in age and symptom of pruritus among them(P<0.05). The levels of ALT, AST, ALP and GGT had significant differences. ANA, AMA, Gp210 and SPIOO were found in AIH -PBC overlap group and the positive rates of AMA, Gp210 and SP100 were remarkably higher than AIH group whereas the rate of SMA was higher than PBC group( P <0.05). The pathological findings in the overlap syndrome showed combined features of AIH and PBC. Whatever UDCA added or not added in glucocorticoids, level of ALP and GGT decreased after therapy. In combined group, the improvement of ALT, AST, GLB and IgG got significance(P<0. 05). Biochemical responses in terms of AIH fea-tures( ALT decline to normal, IgG≤ 16 g/L) were achieved in especially combined group. Although more alleviation of inflammatory infiltration and damage of bile ducts appeared in combined group, adverse reaction such as fracture or alimentary tract hemorrhage were also higher observed in this group. Conclusion AIH - PBC overlap syndrome showed combined features of both PBC and AIH and there were unique characteristic of its own. Combination of UDCA and glucocorticoids is benefit for achieving better biochemical response and pathological improvement for AIH - PBC overlap syndrome.%目的 观察自身免疫性肝炎和原发性胆汁性肝硬化(AIH-PBC)重叠综合征的临床特征及治疗效果.方法 研究1:回顾分析124例PBC、57例AIH、39例AIH-PBC重叠综合征患者的临床特征;研究2:根据不同治疗方案对39例AIH-PBC重叠综合征患者进行分组疗效分析.结果 在220例自身免疫性肝病患者中,AIH-PBC重叠综合征占17.73％.3组患者的性别组成差异无统计学意义,但发病年龄AIH组＜AIH - PBC组与PBC组,乏力、黄疸等症状体征的发生率无差异,但瘙痒症状3组之间差异有统计学意义(P＜0.05).3组间ALT、AST、ALP及GGT水平差异有统计学意义(P＜0.05),多以AIH-PBC重叠组高.AIH -PBC组自身抗体检测可见抗核抗体(ANA)、抗线粒体抗体(AMA) 、Gp210和SP100抗体阳性,后三项抗体检出率明显高于AIH组,抗平滑肌抗体(SMA)检出率显著高于PBC组(P＜0.05).肝组织病理显示,AIH -PBC重叠综合征兼有PBC和AIH的特点.将39例AIH -PBC患者分为单用熊去氧胆酸( UDCA)组和UDCA联合激素治疗组,治疗后两组ALP、GGT水平均较治疗前有所下降,其中GGT改善有统计学意义；UDCA联合激素治疗组ALT、AST水平较治疗前有显著下降,并在GLB、IgG水平的改善上有统计学意义(P＜0.05).不同治疗组在ALT、IgG指标的生化学应答率上差异存在统计学意义(P＜0.05),以联合治疗组应答率高.联合治疗可以有效减轻AIH-PBC重叠综合征患者肝脏炎症纤维化及胆管损害程度,但有骨折及消化道出血等不良反应发生.结论 AIH-PBC重叠综合征表现出AIH与PBC的双重特征,但又不完全是二者的重叠；对于诊断为AIH-PBC重叠综合征的患者,建议积极应用UDCA与糖皮质激素联合治疗,以获得更佳的生化学应答及组织学改善.