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目的:分析急性重症自身免疫性肝炎（AIH）的临床特征及预后。方法:回顾性分析2008-2019年入住空军军医大学第一附属医院的急性重症AIH患者临床资料，根据有无肝病基础分为急性AIH（A-AIH）和慢加急性AIH（AC-AIH），分析患者一般情况、肝生物化学、免疫学、肝组织学特征、激素治疗预后及相关因素。结果:共收集41例患者[女性39例，年龄（54.24±10.55）岁]，丙氨酸转氨酶（ALT）、总胆红素（TBil）明显升高、国际标准化比率（INR）>1.5，肝组织学可见急性小叶炎为急性重症AIH的特点。血清IgG水平（28.36±8.35） g/L，自身抗体抗核抗体（ANA）阳性率82.9%，抗平滑肌抗体（ASMA）17.1%。70%以上急性重症AIH为AC-AIH，AC-AIH发病病程多>8周，而A-AIH多数 1.5. Acute lobular inflammation was the feature of acute and severe AIH in the histology of liver. The serum IgG level was (28.36 ± 8.35) g / L. The positive rate of antinuclear antibody (ANA) and anti-smooth muscle antibody (ASMA) was 82.9%, and 17.1%, respectively. Over 70% of acute severe AIHs were AC-AIH. The duration of onset of AC-AIH was > 8 weeks, while most A-AIHs < 8 weeks, and the differences between the two groups were statistically significant ( n P = 0.001). The mortality rate within 30 days after hormonal treatment was 19.5%. There were statistically significant differences in TBil, Model for End-Stage Liver Disease (MELD) score and leukocyte count between the death and survival group.n Conclusion:The mortality rate in acute severe AIH is high, and most of them have the basis of chronic liver disease. Serum IgG level, autoantibodies and acute lobular inflammation are important factors for diagnosis. The prognosis of hormonal therapy is related to the patients’ condition and course of disease.