关键词： ACLF, acute-on-chronic liver failure AIH, autoimmune hepatitis ALF, acute liver failure ALI, acute liver injury ALT, alanine aminotransferase ANA, anti-nuclear antibody AS-AIH, acute severe autoimmune hepatitis ASMA, anti-smooth muscle antibody AST, aspartate aminotransferase AUROC, analysis of area under the receiver operator characteristic curve Acute liver failure Acute severe presentation Autoimmune hepatitis CT, computed tomography Corticosteroids DILI, drug-induced liver injury EBV, Epstein-Barr virus HE, hepatic encephalopathy HLA, human leukocyte antigen IAIHG, International Autoimmune Hepatitis Group INR, international normalised ratio LT, liver transplantation Liver transplantation MELD, model for end-stage liver disease MELD-Na, model for end-stage liver disease-sodium MHN, massive hepatic necrosis NAC, N-acetylcysteine PT, prothrombin time UKELD, United Kingdom end-stage liver disease USALF, United States Acute Liver Failure anti-LC-1, anti-liver cytosol-1 anti-LKM, anti-liver kidney microsomal anti-SLA/LP, anti-soluble liver antigen/liver pancreas

来  源：   DOI：10.1016/j.jhepr.2020.100149   PDF(Sci-hub)   PDF(Pubmed)

Abstract：
Autoimmune hepatitis is associated with varied clinical presentations and natural history, as well as somewhat unpredictable treatment responses. Understanding how to stratify patients who require further escalation of therapy will help clinicians manage these patients. The presentation of acute severe autoimmune hepatitis (AS-AIH) is relatively uncommon, although its prevalence is potentially greater than currently perceived. Previous studies consist of small retrospective single-centre series and are not directly comparable due to the diversity of presentations, disease definitions and non-standardised treatment regimens. We define AS-AIH as those who present acutely with AIH and are icteric with an international normalised ratio ≥1.5 and no evidence of hepatic encephalopathy. Those with hepatic encephalopathy should be defined as having AS-AIH with acute liver failure. In this review, we provide a structured practical approach for diagnosing and managing this unique group of patients.
摘要：
自身免疫性肝炎与各种临床表现和自然史有关，以及一些不可预测的治疗反应。了解如何对需要进一步升级治疗的患者进行分层将有助于临床医生管理这些患者。急性重症自身免疫性肝炎（AS-AIH）的表现相对少见，尽管其患病率可能高于目前的感知。以前的研究由小型回顾性单中心系列组成，由于演示文稿的多样性，无法直接比较。疾病定义和非标准化治疗方案。我们将AS-AIH定义为AIH急性发作且黄疸，国际标准化比率≥1.5且无肝性脑病证据的患者。肝性脑病患者应定义为AS-AIH伴急性肝衰竭。在这次审查中,我们为诊断和管理这一组独特的患者提供了一种结构化的实用方法.
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