Objective: To analyze the clinical features and prognosis of acute severe autoimmune hepatitis (AIH). Methods: A retrospective analysis of the clinical data of patients with acute severe AIH admitted to our hospital from 2008 to 2019 was divided into acute AIH (A-AIH) and chronic acute AIH (AC-AIH) according to the presence or absence of liver diseases. Patients' general condition, liver biochemistry, immunology, histological features of liver, hormonal therapies prognosis and related factors were analyzed. Results: A total of 41 cases [39 females, age (54.24 ± 10.55) years] were collected. Alanine aminotransferase (ALT) and total bilirubin (TBil) were significantly increased, and the international normalized ratio (INR) was > 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 (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. 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.
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<8周，两组差异有统计学意义（P = 0.001）。激素治疗后30 d内病死率为19.5%，死亡组与生存组之间TBil、终末期肝病模型（MELD）评分和白细胞计数差异有统计学意义。 结论： 急性重症AIH病死率高，多数有慢性肝病基础，血清IgG水平、自身抗体和急性小叶炎为诊断重要因素，激素治疗预后与患者病情及病程有关。.
Keywords: Acute severe hepatitis; Antinuclear antibody; Autoimmune hepatitis; Centrilobular necrosis/inflammation; Corticosteroid.