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Partial Bile Duct Ligation in the Mouse: A Controlled Model of Localized Obstructive Cholestasis

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Primary biliary cirrhosis: From bench to bedside.

Elias Kouroumalis¹, George Notas¹

¹Elias Kouroumalis, Department of Gastroenterogy and Hepatology, University of Crete Medical School, 71003 Heraklion, Crete, Greece.

World Journal of Gastrointestinal Pharmacology and Therapeutics

|August 12, 2015

Summary

Primary biliary cirrhosis (PBC) is an autoimmune liver disease with unknown causes. This review explores epidemiological data, pathogenesis, and treatment, proposing a new theory for PBC development.

Keywords:

Adaptive immunity Chemokines Cytokines Innate immunity Macrophages Primary biliary cirrhosis Ursodeoxycholate

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Area of Science:

Hepatology
Immunology
Gastroenterology

Background:

Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease characterized by non-suppurative destructive intrahepatic cholangitis.
The precise etiology and pathogenesis of PBC remain largely unknown, although autoimmune mechanisms are strongly implicated.

Purpose of the Study:

To review epidemiological and geoepidemiological data on PBC.
To examine the complex pathogenetic aspects, including genetic and environmental factors, and the roles of innate and adaptive immunity.
To present current pathogenetic theories and formulate a novel unifying theory for PBC development.

Main Methods:

Comprehensive review of existing epidemiological, geoepidemiological, and clinical data.
Analysis of in vivo and in vitro studies focusing on the pathophysiology of PBC.
Examination of the roles of genetic background, environmental factors, immune cells, cytokines, chemokines, apoptosis, and reactive oxygen species.

Main Results:

PBC initiation and progression are multifactorial, influenced by genetic and environmental factors.
Innate and adaptive immunity play significant roles, involving cytokines, chemokines, macrophages, apoptosis, and reactive oxygen species.
A novel pathogenetic theory is formulated, integrating clinical, in vitro, and in vivo data.

Conclusions:

Understanding PBC requires considering genetic predisposition, environmental triggers, and complex immune system dysregulation.
Further research into the proposed pathogenetic theory can guide future diagnostic and therapeutic strategies for PBC.
Current treatment modalities, including ursodeoxycholate, are discussed in relation to their mechanisms of action.