不明原因肝硬化的病理诊断思路

张誉; 杨永峰

doi:10.3969/j.issn.1001-5256.2023.03.003

不明原因肝硬化的病理诊断思路

DOI: 10.3969/j.issn.1001-5256.2023.03.003

张誉¹,
杨永峰^2, , ,

1.
东南大学医学院，南京 210009
2.
南京中医药大学附属南京医院(南京市第二医院) 肝病科，南京 210003

基金项目:

江苏省卫健委重点研究项目 (ZD2021061)

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：张誉负责文献检索，撰写文章；杨永峰负责设计文章框架，文献检索策略，修改和审核文章。

详细信息

通信作者:
杨永峰，yyf1997@163.com (ORCID: 0000-0002-3214-0038)

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出版历程
- 收稿日期: 2023-01-16
- 录用日期: 2023-02-20
- 出版日期: 2023-03-20

Thinking of the pathological diagnosis of unexplained liver cirrhosis

Yu ZHANG¹,
Yongfeng YANG^{2
, ,
,}

1.
School of Medicine, Southeast University, Nanjing 210009, China
2.
Department of Hepatology, Nanjing Hospital Affiliated to Nanjing University of Chinese Medicine & Nanjing Second Hospital, Nanjing 210003, China

Research funding:

Jiangsu Commission of Health (ZD2021061)

More Information

Corresponding author: YANG Yongfeng, yyf1997@163.com (ORCID: 0000-0002-3214-0038)

摘要

摘要: 肝硬化是各类慢性肝病发展的终末阶段，常见的病因包括慢性病毒性肝炎、非酒精性脂肪性肝病、自身免疫性肝病、遗传代谢性肝病等。明确病因诊断是对因治疗的重要前提。“不明原因”肝硬化指经过常规病史询问、体格检查及辅助检查后无法明确病因的肝硬化。此时，肝组织病理学检查既是肝硬化诊断的“金标准”，也是探究肝硬化病因的重要依据。通过对肝纤维化模式、炎症损伤类型及伴随病理改变等进行评估，同时结合相关病史、体征、实验室检查及影像学检查结果综合分析，有助于不明原因肝硬化的病因诊断。本文将对不明原因肝硬化的病理特征及诊断思路进行综述。
- 肝硬化 /
- 病理学 /
- 诊断
Abstract: Liver cirrhosis is the final stage of various chronic liver diseases, and the common etiologies of liver cirrhosis include chronic viral hepatitis, nonalcoholic fatty liver disease, autoimmune liver diseases, and inherited metabolic liver disease. An accurate etiological diagnosis is an important prerequisite for etiological treatment. Unexplained liver cirrhosis refers to liver cirrhosis without a definite etiology after medical history inquiry, physical examination, and auxiliary examination. At present, liver histopathological examination is a gold standard for the diagnosis of liver cirrhosis and an important basis for exploring the etiology of liver cirrhosis. It may help with the etiological diagnosis of unexplained liver cirrhosis to evaluate the pattern of liver fibrosis, the type of inflammatory injury, and related pathological changes with reference to a comprehensive analysis of related medical history, signs, laboratory examination, and radiological examination. This article reviews the pathological features and diagnostic thinking of unexplained liver cirrhosis.
- Liver Cirrhosis /
- Pathology /
- Diagnosis

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表 1 NASH肝硬化临床试验中的诊断共识

Table 1. Diagnostic consensus in clinical trials of NASH cirrhosis

明确的	很可能的	可能的
a.当前的肝活检显示肝硬化合并脂肪性肝炎。无竞争性病因¹⁾的证据	a.既往肝活检显示脂肪变性，目前根据病史、体征、影像学检查及肝活检等提示有肝硬化。无竞争性病因¹⁾的证据。至少合并2项代谢异常²⁾或有相关病史，包括肥胖³⁾和/或2型糖尿病，以证实NAFLD的诊断	a.“隐源性肝硬化”且无当前或既往影像学脂肪变性及组织学脂肪变性/脂肪性肝炎的证据。无竞争性病因¹⁾的证据。至少合并1项代谢异常²⁾或有相关病史，包括肥胖³⁾和/或2型糖尿病，以证实NAFLD的诊断
b.既往肝活检显示脂肪性肝炎，目前根据病史、体征、影像学检查及肝活检等提示有肝硬化。无竞争性病因¹⁾的证据。至少合并1项代谢异常²⁾或有相关病史，以证实NAFLD的诊断	b.肝硬化合并当前或既往影像学提示脂肪变性。无肝组织学检查。无竞争性病因¹⁾的证据。同时合并至少2项代谢异常²⁾或有相关病史，包括肥胖³⁾和/或2型糖尿病，以证实NAFLD的诊断	b.既往HCV根除史或大量饮酒史，目前有肝硬化的证据及脂肪性肝炎的组织学证据
c.当前的肝活检显示肝硬化合并脂肪变性(无活动性脂肪肝炎)。无竞争性病因¹⁾的证据。同时合并至少2项代谢异常²⁾或有相关病史，包括肥胖³⁾和/或2型糖尿病，以证实NAFLD的诊断	c.“隐源性肝硬化”且无当前或既往影像学脂肪变性及组织学脂肪变性/脂肪性肝炎的证据。无竞争性病因¹⁾的证据。同时合并至少2项代谢异常²⁾或有相关病史，包括肥胖³⁾和/或2型糖尿病，以证实NAFLD的诊断
注：1)竞争性病因：酒精、病毒性肝炎、血色病、PBC、PSC、肝豆状核变性、α-1-抗胰蛋白酶缺乏症和AIH; 2)代谢异常: 2型糖尿病、高血压病、血脂异常或肥胖症，建议至少5年病史; 3)肥胖：BMI≥30 kg/m²或向心性肥胖。

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