Primary biliary cirrhosis and Sjögren’s syndrome: Autoimmune epithelitis
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Primary biliary cirrhosis (PBC) has been often coined a model autoimmune disease based on the homogeneity amongst patients, the frequency and similarity of antimitochondrial antibodies, including the highly directed immune response to pyruvate dehydrogenase (PDC-E2). A significant number of patients with PBC suffer from sicca and amongst these, there are patients who also have classic Sjögren's syndrome. Indeed, both PBC and Sjögren's syndrome are characterized by inflammation of target epithelial elements. Both diseases can be considered on the basis of a number of other related clinical aspects, including proposed unique apoptotic features of the target tissue, the role of secretory IgA, and the frequency with which both diseases overlap with each other. Indeed, PBC may be considered a Sjögren's syndrome of the liver, whereas Sjögren's syndrome can be equally discussed as PBC of the salivary glands. Dissection of the genetic predispositions for both diseases and especially the molecular basis of effector mechanisms, will become critical elements in developing new therapies.
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Similar anti-mitochondrial antibodies have been reported in Sjögren syndrome patients, raising interest in their pathogenic involvement in diseases affecting exocrine glands and epithelia.
“Notably, several studies have reported the presence of similar anti-mitochondrial antibodies in patients with Sjögren syndrome239,240, raising interest in the pathogenic involvement of anti-mitochondrial antibodies in diseases that affect the exocrine glands and epithelia241.”
From The role of mitochondria in rheumatic diseases (2022) · cites this paper as [241]
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Antinuclear Antibodies in Primary Biliary Cirrhosis
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The X in sex: how autoimmune diseases revolve around sex chromosomes
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Self-citationEpigenetics in autoimmune disorders: Highlights of the 10th Sjögren's Syndrome Symposium
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Small ubiquitin‐related modifiers
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Self-citationAnti-gp210 antibody mirrors disease severity in primary biliary cirrhosis
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Fatigue is not a specific symptom in patients with primary biliary cirrhosis
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Infectious Agents and Xenobiotics in the Etiology of Primary Biliary Cirrhosis
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Self-citationImmunogenetics of systemic sclerosis
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Self-citationT cell clonal expansions detected in patients with primary biliary cirrhosis express CX3CR1
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Self-citationInfectious agents and xenobiotics in the etiology of primary biliary cirrhosis.
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Self-citationAnti-Mitochondrial Antibody–Negative Primary Biliary Cirrhosis
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Self-citationPrimary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes
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Memory B-cell aggregates in skin biopsy are diagnostic for primary Sjögren’s syndrome
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The autoimmunity of primary biliary cirrhosis and the clonal selection theory
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Cutting-Edge Issues in Primary Biliary Cirrhosis
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Self-citationIn situ mass spectrometry of autoimmune liver diseases
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Self-citationReproductive Immunology: a Focus on the Role of Female Sex Hormones and Other Gender-Related Factors
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Self-citationExtrahepatic autoimmune conditions associated with primary biliary cirrhosis
Floreani, A., Franceschet, I., Cazzagon, N., Spinazzè, A., Buja, A., Furlan, P., Baldo, V., & Gershwin, M. E. (2015) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-014-8427-x · Cited by 188 · see citing works
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Tincani, A., Andréoli, L., Cavazzana, I., Doria, A., Favero, M., Fenini, M. G., Franceschini, F., Lojacono, A., Nascimbeni, G., Santoro, A., et al. (2013) · BMC Medicine · doi:10.1186/1741-7015-11-93 · Cited by 165 · see citing works
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Guillain-Barré syndrome: causes, immunopathogenic mechanisms and treatment
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Vitamin D Deficiency, Osteoporosis and Effect on Autoimmune Diseases and Hematopoiesis: A Review
Martinis, M. D., Allegra, A., Sirufo, M., Tonacci, A., Pioggia, G., Raggiunti, M., Ginaldi, L., & Gangemi, S. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22168855 · Cited by 120 · see citing works
Extrahepatic Manifestations of Primary Biliary Cholangitis
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Environmental factors in primary biliary cirrhosis
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- Authors
- Selmi, C., Meroni, P. L., & Gershwin, M. E.
- Affiliations
- University of California, Davis (US)
- University of Milan (IT)
- Venue
- Journal of Autoimmunity
- Publisher
- Elsevier BV
- Published
- 2012-08
- Volume
- 39
- Issue
- 1-2
- Pages
- 34-42
- ISSN
- 0896-8411 (ISSN-L)
- PMC
- PMC3568660
- OpenAlex
- W2066208025
- Cited by
- 145
- References
- 168
- Citations by year
- 2026: 5 · 2025: 7 · 2024: 12 · 2023: 8 · 2022: 10 · 2021: 10 · 2020: 12 · 2019: 6 · 2018: 8 · 2017: 12 · 2016: 8 · 2015: 11 · 2014: 12 · 2013: 15 · 2012: 8
- FWCI
- 1.42
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Journal of Autoimmunity (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (R37DK039588, R01DK039588)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Physiology › Salivary Gland Disorders and Functions
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- primary biliary cirrhosis, Sjögren's syndrome, autoimmune epithelitis, antimitochondrial antibodies, pyruvate dehydrogenase E2, sicca symptoms, secretory IgA, apoptosis, genetic susceptibility, disease overlap
- MeSH terms
- Autoantibodies, Humans, Immunoglobulin A, Secretory, Liver, Liver Cirrhosis, Biliary, Mitochondria, Pyruvate Dehydrogenase Complex, Salivary Glands, Sjogren's Syndrome, Autoimmunity
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