以黄疸为首发表现的自身免疫性肝炎临床病理特点

Clinicopathological features of autoimmune hepatitis patients with jaundice as the initial symptoms

目的分析以黄疸为首发表现的自身免疫性肝炎（AIH）患者与非黄疸AIH患者的临床病理特点。方法纳入2007年6月至2012年7月间共79例AIH患者，其中36例以黄疸为首发表现（黄疸组），43例为非黄疸AIH患者（非黄疸组），比较两组患者临床症状体征、血清生物化学功能指标、免疫学指标及肝活组织检查病理学资料。非正态连续变量的比较使用Mann—WhitneyU检验，分类资料构成比的比较使用卡方检验，治疗应答中累积生物化学缓解率的估计使用Kaplan—Meier法。结果黄疸组、非黄疸组AIH患者的一般特点、免疫学指标、自身抗体阳性率、典型组织病理学特征比较，差异均无统计学意义（P均〉O．05）。但黄疸组AIH患者的肝功能主要指标、肾功能指标、肝组织炎症分级和纤维化分期、胆管反应发生率、肝小叶中央区坏死程度明显高于非黄疸组（P均d0．05）。采用国际自身免疫性肝炎小组（IAIHG）1999年更新的积分诊断评估时，黄疸组治疗前得分低于非黄疸组（14．670±3．347比16．370±2．699；Z=-2．161，P=0．031）；采用简化积分系统评估时，黄疸组得分与非黄疸组差异无统计学意义（6．920±0．874比6．770±1．109；Z=-0．388，P=0．698）。黄疸组中33例接受免疫抑制治疗，累积生物化学缓解率为80．6％，中位缓解时间为26个月；非黄疸组中36例接受免疫抑制治疗，累积生物化学缓解率为94．1％，中位缓解时间为7个月，差异有统计学意义（X2=7．767，P〈0．01）。结论以黄疸为首发表现的AIH患者的临床和肝组织学表现较非黄疸患者更严重，缓解率较低。早期诊断制定合适的治疗方案意义重大。

Objective To explore the clinical and pathological features of icteric and nonicteric patients with autoimmune hepatitis （AIH）. Methods Seventy-nine AIH patients were enrolled in this study. Thirty-six cases were icteric patients and forty-three cases were nonicteric patients. The clinical data serum biochemical indexes, immunological indexes and histological data of the icteric cases were compared with the nonicteric cases. The data were analyzed by Mann-Wbithey U test, chi-square test and Kaplan-Meier estimator. Results There was no significant difference between two groups in the genenal condition, immunological test, autoantibodies test and typical AIH histological findings. However, the icteric AIH patients had more severe changes in biochemical tests, hepatic inflammatory degrees, fibrosis grades, histological ductular reactions and centrilobular necrosis than those of nonicteric patients. The IAIHG＇s revised original scoring system pretreatment scores of icteric patients were lower than that of nonicteric patients （14. 670±3. 347 vs 16. 370±2. 699; Z= -2. 161, P= 0. 031）, whereas there was no significant difference in the simplified scoring system scores between two groups （6. 920±0. 874 vs 6. 770±1. 109; X2=0.388, P=0.698）. There were 33 icteric AIH patients who received immunosuppressive therapy. The cumulative biochemical remission rate in icteric AIH cases was 80.6% with 26 months of median duration of remission, whereas cumulative biochemical remission rate in icteric AIH cases was 94. 1% with 7 months of median duration of remission （Z2 =7. 767, P〈0. 01）. Conclusions The icteric AIH patients tend to have more severe clinical and histological presentations and lower remission rate than nonicteric patients. Early diagnosis and appropriate therapeutic measures are of great significance.