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Primary biliary cirrhosis and Sjögren’s syndrome: Autoimmune epithelitis
Open accessAbstract
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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“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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Metadata
- 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 (medical) › 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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