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Updated: Feb 28, 2026
Induction of Drug-Induced, Autoimmune Hepatitis in BALB/c Mice for the Study of Its Pathogenic Mechanisms
Published on: May 29, 2020
Autoimmune cholangiopathy
1Center for Liver Diseases and Transplantation, Los Angeles, California, USA. vierling@csmc.edu
Insights
Current evidence is insufficient to distinguish autoimmune cholangitis (AIC) from primary biliary cholangitis (PBC). Further research into causes and mechanisms is needed to determine if AIC is a separate disease from AMA-positive PBC.
Area of Science:
- Hepatology
- Immunology
- Gastroenterology
Background:
- Primary biliary cholangitis (PBC) is an autoimmune liver disease.
- Autoimmune cholangitis (AIC) is defined as AMA-negative PBC with ANA or SMA.
- The distinction between PBC and AIC remains unclear.
Purpose of the Study:
- To evaluate the current evidence regarding the classification of PBC and AIC.
- To discuss the potential for AIC to be recognized as a distinct disease entity.
Main Methods:
- Review of existing scientific literature and evidence.
- Analysis of diagnostic criteria and serological markers (AMA, ANA, SMA).
Main Results:
- Current evidence is insufficient to definitively classify AIC as a separate disease from PBC.
- Further investigation into etiological factors and pathogenetic mechanisms is required.
Conclusions:
- The term "autoimmune cholangitis" has been proposed for PBC with or without AMA.
- PBC is likely to remain the established term, with AIC and AMA-negative PBC used interchangeably by hepatologists.
Abstract:
Currently available evidence is insufficient to classify PBC and AIC as separate diseases. The ultimate answer to the question of whether AIC, defined as AMA-negative PBC with ANA or SMA, is a disease distinct from AMA-positive PBC with or without ANA will require a detailed comparison of etiologic factors and pathogenetic mechanisms, once they are elucidated. It is intriguing to consider the suggestion of Heathcote that the term autoimmune cholangitis be adopted to describe PBC with or without detectable AMA. However, it is improbable that the venerable term PBC will be supplanted. Hepatologists will probably continue to use the terms AIC and AMA-negative PBC interchangeably, with little risk of being misunderstood.
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