原发性胆汁性胆管炎诊治——病理是否必需？

刘红丽; 杨永峰

doi:10.3969/j.issn.1001-5256.2023.03.005

原发性胆汁性胆管炎诊治——病理是否必需？

DOI: 10.3969/j.issn.1001-5256.2023.03.005

刘红丽^{1, 2},
杨永峰^{2, 3, , ,}

1.
东南大学医学院，南京 210009
2.
东南大学教学医院南京市第二医院肝病科，南京 210003
3.
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:

国家自然科学基金 (81970454)

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：刘红丽负责检索文献，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: 2022-12-31
- 录用日期: 2023-02-17
- 出版日期: 2023-03-20

Diagnosis and treatment of primary biliary cholangitis: Is pathology necessary?

Hongli LIU^{1, 2},
Yongfeng YANG^{2, 3
, ,
,}

1.
School of Medicine, Southeast University, Nanjing 210009, China
2.
Department of Hepatology, Nanjing Second Hospital & Teaching Hospital of Southeast University, Nanjing 210003, China
3.
Department of Hepatology, Nanjing Hospital Affiliated to Nanjing University of Chinese Medicine & Nanjing Second Hospital, Nanjing 210003, China

Research funding:

National Natural Science Foundation of China (81970454)

More Information

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

摘要

摘要: 原发性胆汁性胆管炎(PBC)是一种慢性肝内胆汁淤积性疾病。本文归纳了PBC病理组织学特征、病理检查在PBC的诊断和治疗中的作用，对病理在分期预后、不典型PBC的诊断、重叠综合征诊断、熊去氧胆酸UDCA应答不佳原因分析和鉴别疾病或排除其他合并疾病等中的作用进行综述，提高临床医生对病理检查对PBC作用的认识。
- 肝硬化, 胆汁性 /
- 病理学, 临床 /
- 诊断 /
- 治疗学
Abstract: Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease. This article summarizes and reviews the histopathological features of PBC and the role of pathological examination in the diagnosis and treatment of PBC, as well as the role of pathology in staging and prognosis, the diagnosis of atypical PBC and overlap syndrome, the analysis of reasons for poor response to ursodeoxycholic acid, and identification of diseases or exclusion of other comorbidities, so as to improve the awareness of the role of pathological examination in PBC among clinicians.
- Liver Cirrhosis, Biliary /
- Pathology, Clinical /
- Diagnosis /
- Therapeutics

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图 1 PBC病理特征

注：a, 淋巴滤泡+胆管损伤+肉芽肿(HE染色, ×100)；b, 胆管缺失(HE染色, ×400)；c, 重度界面炎(HE染色, ×100)；d, 淋巴滤泡+损伤胆管+胆管反应(免疫组织化学染色CK7, ×200)；e, 汇管区周边胆管反应(免疫组织化学染色CK7，×100)；f，CK7阳性肝细胞(免疫组织化学染色CK7, ×100)。

Figure 1. Pathological features of primary biliary choloangitis

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表 1 Scheuers和Ludwig PBC分期

Table 1. Scheuers and Ludwing stages of primary biliary cholangitis

分期	Scheuer分期	Ludwig分期
Ⅰ期	胆管损伤，胆管明显损伤(汇管区炎症)	汇管区炎
Ⅱ期	细胆管增生(汇管区周围扩张和炎症)	汇管区周围炎，即界面炎
Ⅲ期	桥接瘢痕，纤维隔	桥接坏死、纤维化
Ⅳ期	肝硬化	肝硬化

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表 2 Nakanuma分期及分级

Table 2. Nakanuma stage and grade of primary biliary cholangitis

项目	表现
分期分数
纤维化
0分	无纤维化或仅汇管区纤维化
1分	汇管区纤维化向小叶延伸，偶见不完全相连的纤维间隔
2分	完全相连的纤维间隔伴小叶结构的紊乱
3分	假小叶形成肝硬化
胆管缺失
0分	汇管区小叶内胆管可分辨
1分	＜1/3汇管区出现胆管缺失
2分	1/3~2/3汇管区出现胆管缺失
3分	＞2/3汇管区出现胆管缺失
地衣红阳性颗粒的沉积
0分	无地衣红阳性颗粒沉积
1分	＜1/3的汇管区周围肝细胞有颗粒沉积
2分	1/3~2/3的汇管区周围肝细胞有颗粒沉积
3分	＞2/3的汇管区周围肝细胞有颗粒沉积
分期¹⁾
0分/0分	1期
1~2分/1~3分	2期(轻度进展)
3~4分/4~6分	3期(中度进展)
5~6分/7~9分	4期(重度进展)
病理分级
CA
CA0	无活动：无胆管炎，但胆管上皮细胞可轻度损伤
CA1	轻度活动：一个受损的胆管为明显的慢性胆管炎(受损的胆管完全被轻度-中度的淋巴-浆细胞包围)
CA2	中度活动：超过两个胆管伴有明显的慢性胆管炎
CA3	重度活动：至少有一个受损的胆管表现为慢性非化脓性胆管炎(胆管上皮细胞受损，胆管完全被大量的淋巴－浆细胞浸润并被上皮样肉芽肿包围)
HA
HA0	无活动：无界面肝炎，且小叶性肝炎无或最轻微
HA1	轻度活动：界面肝炎累及一个汇管区或纤维间隔周围连续10个肝细胞，且有轻度-中度小叶性肝炎
HA2	中度活动：界面肝炎累及两个以上汇管区或纤维间隔周围连续10个肝细胞，且有轻度-中度小叶性肝炎
HA3	重度活动：界面肝炎累及一半以上的汇管区周围连续20个肝细胞，且有中度小叶性肝炎或桥接或带状坏死
注：1)，(纤维化+胆管缺失)/(纤维化+胆管缺失+地衣红阳性颗粒沉积)。CA，胆管活动；HA，肝炎活动。

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