重视对急性重症自身免疫性肝炎的诊治

连敏; 唐茹琦; 马雄

doi:10.12449/JCH250705

重视对急性重症自身免疫性肝炎的诊治

DOI: 10.12449/JCH250705

连敏^a,
唐茹琦^a,
马雄^{a, b, , ,}

a.
上海交通大学医学院附属仁济医院消化内科/上海市消化疾病研究所，上海 200001
b.
上海交通大学医学院附属仁济医院衰老与组织修复研究院，上海 200001

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：连敏负责查阅资料及文章撰写；唐茹琦、马雄负责对文章内容作批评性审阅，指导文章方向及最后定稿。

详细信息

通信作者:
马雄， maxiongmd@163.com （ORCID： 0000-0001-9616-4672）

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出版历程
- 收稿日期: 2025-04-16
- 录用日期: 2025-05-29
- 出版日期: 2025-07-25

Emphasizing the diagnosis and treatment of acute severe autoimmune hepatitis

Min LIAN^a,
Ruqi TANG^a,
Xiong MA^{a, b
, ,
,}

a.
Department of Gastroenterology/Shanghai Institute of Digestive Disease, Renji Hospital，Shanghai Jiao Tong University School of Medicine，Shanghai 200001，China
b.
Institute of Aging & Tissue Regeneration, Renji Hospital，Shanghai Jiao Tong University School of Medicine，Shanghai 200001，China

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Corresponding author: MA Xiong， maxiongmd@163.com （ORCID： 0000-0001-9616-4672）

摘要

摘要: 目前，急性重症自身免疫性肝炎（AIH）的诊断标准包括急性起病、符合AIH诊断标准，且诊断时存在黄疸、INR≥1.5、尚未出现肝性脑病以及无既往肝病证据。作为AIH的一类特殊亚型，急性重症AIH具有起病急、病情进展迅速的特点，早期诊断识别至关重要，一经诊断应及早给予糖皮质激素治疗，可降低患者进展至肝衰竭乃至接受肝移植的风险。该病的临床管理极具挑战性，诊断时患者往往缺少典型AIH的临床特征和组织学表现，早期评估激素应答反应，并给予适当剂量的治疗方案对于改善患者预后至关重要。
- 肝炎，自身免疫性 /
- 糖皮质激素类 /
- 肝移植
Abstract: At present， the diagnostic criteria for acute severe autoimmune hepatitis （AS-AIH） include acute onset， consistency with the diagnostic criteria for autoimmune hepatitis （AIH）， presence of jaundice， and international normalized ratio ≥1.5 at the time of diagnosis， without evidence of hepatic encephalopathy or previous liver disease. As a special subtype of AIH， AS-AIH is characterized by acute onset and rapid disease progression， and thus early diagnosis is of vital importance. Glucocorticoid therapy should be given as early as possible after diagnosis to prevent the progression to acute liver failure and reduce the risk of liver transplantation. Management of AS-AIH patients is challenging， since patients often lack typical clinical features and histological manifestations of AIH at the time of diagnosis， and early assessment of glucocorticoid response and a treatment regimen with proper doses are important for improving the prognosis of patients.
- Hepatitis， Autoimmune /
- Glucocorticoids /
- Liver Transplantation

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表 1 AS-AIH的代表性队列

Table 1. Representative cohort of AS-AIH

研究者	病例数	定义	胆红素	INR	MELD （分）	激素治疗［例（%）］	激素剂量	治疗前 HE ［例（%）］	激素治疗组预后
de Martin 等^［13］， 2021年	128	急性表现 AIH组织学表现（90%） INR≥1.5±胆红素>200 mmol/L 未排除进展性肝纤维化或肝硬化	259 （197～400） mmol/L	1.8 （1.6～2.6）	25 （21～28）	115 （90）	73%： 1 mg·kg^-1·d^-1 27%： 0.5 mg·kg^-1·d^-1	23 （18）	76例（66%）激素应答，30例肝移植，12例死亡
Téllez 等^［15］， 2021年	242	急性表现（<3个月） INR≥1.5 无慢性肝病史符合IAIHG简化评分	平均14.5（ 8.7）mg/dL	平均2.1 （0.8）	平均24 （5）	203 （83.9）	79.8%： 1 mg·kg^-1·d^-1	33 （13.6）	141例（69.5%）激素应答，32例肝移植，36例死亡
Yeoman 等^［11］， 2014年	32	急性表现（<26周） INR≥1.5 无慢性肝炎组织学病变	463 （55～1 208） μmol/L	2.2 （1.5～3.5）	29（ 22～40）	23 （72）	20～40 mg/d （口服）或氢化可的松100 mg、 3次/d（静脉注射）	5/23 （21） 1～2级	10例（43%）激素应答，另2例对免疫抑制剂应答， 10例肝移植
Wang 等^［16］， 2025年	97	急性起病（<30 d）胆红素>5 mg/dL INR≥1.5 无肝性脑病无慢性肝病史	186 （123～277） μmol/L	1.5 （1.5～1.7）	16 （15～18）	90 （93）	诱导方案： 20～60 mg/d	0	68例（75%）激素应答，4例肝移植，6例死亡
Zachou 等^［17］， 2019年	34	急性肝炎（<24周）无HE 转氨酶>10倍正常值上限胆红素>4 mg/dL INR≥1.5 无慢性肝炎组织学病变	173 （54～619） mmol/L	1.52 （1.50～2.27）	18 （12～24）	34 （100）	诱导方案： 1.5 mg·kg^-1·d^-1	0	33例（97%）激素应答，1例死亡

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