187例自身免疫性肝病的临床特点分析

目的分析自身免疫性肝病（AILD）的临床、生化及免疫学特点。方法分析北京大学人民医院2001年1月～2010年12月187例自身免疫性肝病患者的临床表现、生化及免疫学特点。结果 AILD的发病以40岁以上女性多见,30.5%（57/187）的患者确诊时已进展为肝硬化失代偿期。临床症状无特异性,自身免疫性肝炎（AIH）以ALT、IgG及γ球蛋白升高更为明显（P〈0.05）,而PBC以ALP、GGT、IgM升高更为明显（P〈0.05）。AILD患者常合并一种或多种肝外自身免疫病,AILD合并干燥综合症最为常见,AIH组及PBC组合并干燥综合症的发生率均达30%以上。AILD患者均有一种或多种自身抗体阳性,其中AIH组ANA阳性率为88.3%,AMA阳性率为7.5%（3/40）,AMA-M2阳性3.7%（2/54）;PBC组ANA阳性率为88.2%（97/110）,AMA阳性率为96.4%（81/84）,AMA-M2阳性率为94.4%（85/90）。AIH患者主要应用糖皮质激素治疗,其中共46例（59.7%）患者单用或联用UDCA治疗后肝脏酶学指标改善。结论生化、免疫学、自身抗体等检查对诊断与鉴别诊断具有重要意义。UDCA在AIH的治疗中有一定的作用。

Clinical analysis of 187 patients with autoimmune liver diseases

Objective To analyze the clinical,biochemical and immunological characteristics of autoimmune liver disease（AILD）.Methods The clinical,biochemical and immunological features of 187 patients with AILD were analyzed.Results AILD mainly affects 40 years old and older female patients.The clinical features are not typical.AIH is characterized by high γ-globulin,alanine aminotransferase（ALT） and immunoglobulin G,but PBC is characterized by high alkaline phosphatase（ALP）,γ-glutamy transpeptidase（GGT） and immunoglobubin M.AILD patients will combine with one or multiple CTD,SS is common,more than 30%.88% of patients with AILD presented significant titers of antinuclear antibody（ANA）,and the positive rate of AMA,AMA-M2 was 96.4% and 94.4% in PBC patients,respectively.AIH patients mainly treated with corticosteroid,and 46 receive UDCA monotherapy or a combination of UDCA and corticosteroid or immunosuppressive agents.Conclusion The patients with autoimmune liver diseases could be diagnosed by biochemical,immunological features and detection of autoantibodies.UDCA is effective in patients with AIH.