Making Sense of Autoantibodies in Cholestatic Liver Diseases
Abstract
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the most common chronic cholestatic liver diseases (CLD) in adults and are associated with immune mechanisms. PBC is considered a model autoimmune disease, and more than 90% of patients present very specific autoantibodies against mitochondrial antigens. Whether PSC should be considered an autoimmune or merely immune-mediated disease is still under debate. This review addresses the clinical relevance of autoantibodies in CLD and their pathogenic mechanisms and illustrates the technology available for appropriate autoantibody detection.
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PBC is characterised serologically by autoantibodies specific for mitochondrial, nuclear and centromere antigens, some of which are unique to PBC.
“PBC is characterised serologically by autoantibodies specific for mitochondrial, nuclear and centromere antigens, some of which are unique to PBC.66–78”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [66]
They are present in ~95% (mitochondrial) and ~30% (nuclear) of patients.
“They are present in ~95% (mitochondrial) and ~30% (nuclear) of patients.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [66]
Unlike in many other autoimmune diseases, these autoantibodies are, as a result of their sensitivity and specificity, extremely useful in diagnosis and have contributed significantly to the decline in the need for liver biopsy, at least for the purposes of diagnosis.
“Unlike in many other autoimmune diseases, these autoantibodies are, as a result of their sensitivity and specificity, extremely useful in diagnosis and have contributed significantly to the decline in the need for liver biopsy, at least for the purposes of diagnosis.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [66]
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References 103 · Cited by 23
- Authors
- Marzorati, S., Invernizzi, P., & Lleo, A.
- Affiliations
- Fondazione Humanitas per la Ricerca (IT)
- Venue
- Clinics in Liver Disease
- Publisher
- Elsevier BV
- Published
- 2016-02
- Volume
- 20
- Issue
- 1
- Pages
- 33-46
- ISSN
- 1089-3261 (ISSN-L)
- OpenAlex
- W2218062651
- Cited by
- 23
- References
- 103
- Citations by year
- 2025: 1 · 2024: 1 · 2023: 3 · 2022: 1 · 2021: 2 · 2020: 4 · 2019: 3 · 2018: 5 · 2017: 3
- FWCI
- 0.38
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Clinics in Liver Disease (publishedVersion)
- PubMed (publishedVersion)
- BOA (University of Milano-Bicocca) (submittedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- autoantibodies, primary biliary cirrhosis, primary sclerosing cholangitis, chronic cholestatic liver disease, mitochondrial antigens, autoimmunity, immune-mediated diseases, autoantibody detection, pathophysiological mechanisms
- MeSH terms
- Antibodies, Antinuclear, Autoantibodies, Autoantigens, Autoimmune Diseases, Enzyme-Linked Immunosorbent Assay, Humans, Liver Cirrhosis, Biliary, Cholangitis, Sclerosing, Fluorescent Antibody Technique, Indirect, Mitochondrial Proteins, Dihydrolipoyllysine-Residue Acetyltransferase
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