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Current Hepatology Reports
Erschienen in: Current Hepatology Reports 2/2020

21.04.2020 | Autoimmune, Cholestatic, and Biliary Diseases (S Gordon and C Bowlus, Section Editors)

Mechanisms of Fibrosis in Primary Biliary Cholangitis

verfasst von: Ling Wu, Jia Ding, Ning-Ping Zhang, Feng Li, Xiu-Ping Liu, Jian Wu

Erschienen in: Current Hepatology Reports | Ausgabe 2/2020

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Abstract

Background

Primary biliary cholangitis (PBC) is an autoimmune liver disease featured with bile duct injury, ductopenia and proliferation, periportal inflammation and fibrosis. Clinical manifestations of PBC vary from almost no symptoms to different degrees of pruritus plus symptoms of liver dysfunction.

Purpose of Review

This review intends to update our understanding in the mechanisms of hepatic fibrogenesis under chronic biliary injury.

Recent Findings

Underlying mechanisms are proposed for a better understanding and more effective therapeutic targets. With genetic predisposition, lesions from bile ducts cause damage to biliary epithelial cells (BECs); and simultaneous autoimmunity reinforces a vicious cycle of BEC damage, inflammatory infiltration, BEC proliferation and fibrogenic responses. Therapeutic efficacy of immunosuppressive agents has been unsatisfactory.

Summary

As a major variable in PBC progression, fibrosis has been paid a great deal of attention but has not been responsive to various treatments.
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Metadaten
Titel
Mechanisms of Fibrosis in Primary Biliary Cholangitis
verfasst von
Ling Wu
Jia Ding
Ning-Ping Zhang
Feng Li
Xiu-Ping Liu
Jian Wu
Publikationsdatum
21.04.2020
Verlag
Springer US
Erschienen in
Current Hepatology Reports / Ausgabe 2/2020
Elektronische ISSN: 2195-9595
DOI
https://doi.org/10.1007/s11901-020-00512-2

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