目的　从病毒标志物均阴性的肝功能异常患者中筛选Ⅰ型自身免疫性肝炎 (AIH)病例 ,并探讨其临床病理表现特征。方法　采用国际自身免疫性肝炎小组 (IAIHG)新修订的描述性诊断标准和计分系统 ,对病毒标志物均阴性的肝功能异常患者进行筛选 ,对符合诊断的患者进行临床病理特点分析。结果　符合诊断的 32例Ⅰ型AIH患者均为女性 ,高发年龄为 4 0～ 6 0岁 ,肝功能生化检查表现为肝炎样改变。 2 8例 (87.5 % )表现为高γ 球蛋白血症 ,2 6例 (81.3% )IgG高于正常。 2 6例 (81.3% )抗核抗体 (ANA)阳性。伴发相关的自身免疫性疾病 12例 (37.5 % )。在有肝穿刺活检组织资料的 15例患者中 ,11例 (73.3% )出现界板性肝炎 ,小叶性肝炎 5例 (33.3% ) ,玫瑰花结样改变 3例 (2 0 .0 % ) ,肝纤维化或肝硬化 6例 (40 .0 % )。 2 1例 (6 5 .6 % )患者对免疫抑制治疗完全应答 ,9例 (2 8.1% )出现复发 ,12例(37.5 % )持续缓解。结论　Ⅰ型AIH患者多为中年女性 ,以肝组织活检呈界板性肝炎、血清自身抗体、高γ 球蛋白血症和对免疫抑制治疗反应良好为特点 Objective To screen patients with type 1 autoimmune hepatitis (AIH) from patients with abnormal hepatic dysfunction who have negative viral markers and investigate the clinical and pathological features. Methods According to the newly revised descriptive diagnostic criteria and scoring system of International Autoimmune Hepatitis Group (IAIHG), all patients with abnormal liver function were screened. All the patients were diagnosed according to the clinicopathological features. Results All 32 cases of type Ⅰ AIH patients were diagnosed as female, with a high incidence of 40 ~ 60 years. The liver biochemical tests showed hepatitis-like changes. Twenty-eight (87.5%) showed hypergammaglobulinemia and 26 (81.3%) had IgG above normal. 26 (81.3%) were positive for antinuclear antibody (ANA). Associated with autoimmune diseases in 12 cases (37.5%). Of the 15 patients with liver biopsy, 11 (73.3%) developed borderline hepatitis, 5 (33.3%) had lobular hepatitis, 3 had rosette-like changes (20.0%), Liver fibrosis or cirrhosis in 6 cases (40.0%). Twenty-one patients (65.6%) responded completely to immunosuppressive therapy, and 9 (21 8.1%) relapsed and 12 (37.5%) sustained remission. Conclusions Type I AIH patients are mostly middle-aged women, characterized by liver biopsy showed de novo hepatitis B, serum autoantibodies, hypergammaglobulinemia and good response to immunosuppressive therapy