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

Hongli LIU; Yongfeng YANG

doi:10.3969/j.issn.1001-5256.2023.03.005

Volume 39 Issue 3

Mar. 2023

Turn off MathJax

Article Contents

Article Navigation > Journal of Clinical Hepatology > 2023 > 39(3): 511-516

PDF( 2564 KB)

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

DOI: 10.3969/j.issn.1001-5256.2023.03.005

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)
Received Date: 2022-12-31
Accepted Date: 2023-02-17
Published Date: 2023-03-20

Abstract

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

FullText(HTML)

References(26)

References

[1]	LV T, CHEN S, LI M, et al. Regional variation and temporal trend of primary biliary cholangitis epidemiology: A systematic review and meta-analysis[J]. J Gastroenterol Hepatol, 2021, 36(6): 1423-1434. DOI: 10.1111/jgh.15329.
[2]	Chinese Society of Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of primary biliary cholangitis (2021)[J]. J Clin Hepatol, 2022, 38(1): 35-41. DOI: 10.3760/cma.j.cn112138-20211112-00794. 中华医学会肝病学分会. 原发性胆汁性胆管炎的诊断和治疗指南(2021)[J]. 临床肝胆病杂志, 2022, 38(1): 35-41. DOI: 10.3760/cma.j.cn112138-20211112-00794.
[3]	GRANITO A, MURATORI P, QUARNETI C, et al. Antinuclear antibodies as ancillary markers in primary biliary cirrhosis[J]. Expert Rev Mol Diagn, 2012, 12(1): 65-74. DOI: 10.1586/erm.11.82.
[4]	ZHANG Q, LIU Z, WU S, et al. Meta-analysis of antinuclear antibodies in the diagnosis of antimitochondrial antibody-negative primary biliary cholangitis[J]. Gastroenterol Res Pract, 2019, 2019: 8959103. DOI: 10.1155/2019/8959103.
[5]	GRANITO A, MURATORI P, MURATORI L, et al. Antinuclear antibodies giving the 'multiple nuclear dots' or the 'rim-like/membranous' patterns: diagnostic accuracy for primary biliary cirrhosis[J]. Aliment Pharmacol Ther, 2006, 24(11-12): 1575-1583. DOI: 10.1111/j.1365-2036.2006.03172.x.
[6]	LUDWIG J, DICKSON ER, MCDONALD GS. Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis)[J]. Virchows Arch A Pathol Anat Histol, 1978, 379(2): 103-112. DOI: 10.1007/BF00432479.
[7]	IMAM MH, LINDOR KD. The natural history of primary biliary cirrhosis[J]. Semin Liver Dis, 2014, 34(3): 329-333. DOI: 10.1055/s-0034-1383731.
[8]	SCHEUER P. Primary biliary cirrhosis[J]. Proc R Soc Med, 1967, 60(12): 1257-1260.
[9]	NAKANUMA Y, ZEN Y, HARADA K, et al. Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: Interobserver agreement[J]. Pathol Int, 2010, 60(3): 167-174. DOI: 10.1111/j.1440-1827.2009.02500.x.
[10]	KAKUDA Y, HARADA K, SAWADA-KITAMURA S, et al. Evaluation of a new histologic staging and grading system for primary biliary cirrhosis in comparison with classical systems[J]. Hum Pathol, 2013, 44(6): 1107-1117. DOI: 10.1016/j.humpath.2012.09.017.
[11]	CARBONE M, NARDI A, FLACK S, et al. Pretreatment prediction of response to ursodeoxycholic acid in primary biliary cholangitis: development and validation of the UDCA Response Score[J]. Lancet Gastroenterol Hepatol, 2018, 3(9): 626-634. DOI: 10.1016/S2468-1253(18)30163-8.
[12]	NORMAN GL, YANG CY, OSTENDORFF HP, et al. Anti-kelch-ike 12 and anti-hexokinase 1: novel autoantibodies in primary biliary cirrhosis[J]. Liver Int, 2015, 35(2): 642-651. DOI: 10.1111/liv.12690.
[13]	DAHLQVIST G, GAOUAR F, CARRAT F, et al. Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis[J]. Hepatology, 2017, 65(1): 152-163. DOI: 10.1002/hep.28859.
[14]	SUN C, XIAO X, YAN L, et al. Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase: Is alkaline phosphatase truly a surrogate marker?[J]. J Autoimmun, 2019, 99: 33-38. DOI: 10.1016/j.jaut.2019.01.005.
[15]	WANG Q, SELMI C, ZHOU X, et al. Epigenetic considerations and the clinical reevaluation of the overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis[J]. J Autoimmun, 2013, 41: 140-145. DOI: 10.1016/j.jaut.2012.10.004.
[16]	MAGO S, WU GY. Primary sclerosing cholangitis and primary biliary cirrhosis overlap syndrome: A review[J]. J Clin Transl Hepatol, 2020, 8(3): 336-346. DOI: 10.14218/JCTH.2020.00036.
[17]	SUNDARAM S, S K, MAZUMDAR S, et al. Overlap syndrome between primary biliary cholangitis and primary sclerosing cholangitis[J]. ACG Case Rep J, 2018, 5: e54. DOI: 10.14309/crj.2018.54.
[18]	MANDOLESI D, LENZI M, D'ERRICO A, et al. Primary biliary cholangitis-primary sclerosing cholangitis in an evolving overlap syndrome: A case report[J]. Gastroenterol Hepatol, 2017, 40(10): 669-671. DOI: 10.1016/j.gastrohep.2016.11.010.
[19]	KINGHAM JG, ABBASI A. Co-existence of primary biliary cirrhosis and primary sclerosing cholangitis: a rare overlap syndrome put in perspective[J]. Eur J Gastroenterol Hepatol, 2005, 17(10): 1077-1080. DOI: 10.1097/00042737-200510000-00011.
[20]	Chinese Society of Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)[J]. J Clin Hepatol, 2022, 38(1): 50-61. DOI: 10.3760/cma.j.cn112138-20211109-00786. 中华医学会肝病学分会. 原发性硬化性胆管炎诊断及治疗指南(2021)[J]. 临床肝胆病杂志, 2022, 38(1): 50-61. DOI: 10.3760/cma.j.cn112138-20211109-00786.
[21]	European Association for the Study of the Liver. EASL clinical practice guidelines: The diagnosis and management of patients with primary biliary cholangitis[J]. J Hepatol, 2017, 67(1): 145-172. DOI: 10.1016/j.jhep.2017.03.022.
[22]	LINDOR KD, BOWLUS CL, BOYER J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases[J]. Hepatology, 2019, 69(1): 394-419. DOI: 10.1002/hep.30145.
[23]	CAREY EJ, ALI AH, LINDOR KD. Primary biliary cirrhosis[J]. Lancet, 2015, 386(10003): 1565-1575. DOI: 10.1016/S0140-6736(15)00154-3.
[24]	ZHANG Y, LI S, HE L, et al. Combination therapy of fenofibrate and ursodeoxycholic acid in patients with primary biliary cirrhosis who respond incompletely to UDCA monotherapy: a meta-analysis[J]. Drug Des Devel Ther, 2015, 9: 2757-2766. DOI: 10.2147/DDDT.S79837.
[25]	TRAUNER M, NEVENS F, SHIFFMAN ML, et al. Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study[J]. Lancet Gastroenterol Hepatol, 2019, 4(6): 445-453. DOI: 10.1016/S2468-1253(19)30094-9.
[26]	YANG J, YU YL, JIN Y, et al. Clinical characteristics of drug-induced liver injury and primary biliary cirrhosis[J]. World J Gastroenterol, 2016, 22(33): 7579-7586. DOI: 10.3748/wjg.v22.i33.7579.

Relative Articles

Supplements(0)

Cited By

Proportional views

Proportional views

通讯作者: 陈斌, bchen63@163.com

1.
沈阳化工大学材料科学与工程学院沈阳 110142

Figures(1) / Tables(2)

Get Citation

PDF

XML

Article Metrics

Article views (694) PDF downloads(205)

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

DOI: 10.3969/j.issn.1001-5256.2023.03.005

Abstract

References

Proportional views

Catalog

通讯作者: 陈斌, bchen63@163.com

Article Metrics

Proportional views

Related

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

DOI: 10.3969/j.issn.1001-5256.2023.03.005

Abstract

References

Proportional views

Catalog

通讯作者: 陈斌, bchen63@163.com

Article Metrics

Proportional views

Related

About Journal

Submission Guideline

Journal Online

Hepatobiliary College

Guidelines

Export File

Citation

Format

Content