| Pathogenesis of Sjögren's syndrome. | |
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MedLine Citation:
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PMID: 19568172 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE OF REVIEW: To summarize recent developments in our understanding of the pathogenesis of Sjögren's syndrome with a focus on the relationship between inflammation and exocrine dysfunction. RECENT FINDINGS: Animal models demonstrated the complex interactions between immunologic and nonimmunologic mechanisms in Sjögren's syndrome. Activation of the innate immune system can lead to exocrine dysfunction before or without significant inflammation, whereas in other models, salivary gland function is preserved despite intense inflammatory infiltrates. Primary or inflammation-related abnormalities in water channels contribute to the exocrinopathy. Activation of the innate immunity in patients is demonstrated by the upregulation of type-1 interferon-regulated genes (interferon signature) in peripheral blood and salivary glands and abnormal expression of B cell-activating factor and its receptors. Nonimmune mechanisms that may contribute to exocrine dysfunction include local and systemic androgen deficiency and autonomic nervous system dysfunction. Autoantibodies against the muscarinic acetylcholine receptors would provide a link between autoimmunity and exocrine dysfunction, but the data on the presence, frequency and physiologic affect of these antibodies remain controversial. SUMMARY: Recent discoveries from studies in patients with Sjögren's syndrome and animal models suggest a complex interplay between genetic factors, environmental and stochastic events that involve innate and adaptive immunity, hormonal mechanisms and the autonomic nervous system. Some of these findings suggest that exocrine gland dysfunction may precede autoimmunity or represent a process independent from inflammation in the pathogenesis of Sjögren's syndrome. |
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Authors:
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Nikolay P Nikolov; Gabor G Illei |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Intramural; Review |
Journal Detail:
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Title: Current opinion in rheumatology Volume: 21 ISSN: 1531-6963 ISO Abbreviation: Curr Opin Rheumatol Publication Date: 2009 Sep |
Date Detail:
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Created Date: 2009-08-13 Completed Date: 2009-11-04 Revised Date: 2012-03-08 |
Medline Journal Info:
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Nlm Unique ID: 9000851 Medline TA: Curr Opin Rheumatol Country: United States |
Other Details:
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Languages: eng Pagination: 465-70 Citation Subset: IM |
Affiliation:
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Sjögren's Syndrome Clinic, National Institute of Dental and Craniofacial Research (NIDCR), Molecular Physiology and Therapeutics Branch, National Institutes of Health (NIH), Bethesda, Maryland 20892, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Autoantibodies / metabolism Autonomic Nervous System / physiopathology B-Cell Activating Factor / metabolism Causality Disease Models, Animal Female Humans Interferon-alpha / genetics, metabolism Male Receptors, Muscarinic / immunology Sex Characteristics Sjogren's Syndrome / etiology*, genetics, immunology, physiopathology |
| Grant Support | |
ID/Acronym/Agency:
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Z01 DE000704-06/DE/NIDCR NIH HHS; Z01 DE000704-07/DE/NIDCR NIH HHS; Z99 DE999999/DE/NIDCR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Autoantibodies; 0/B-Cell Activating Factor; 0/Interferon-alpha; 0/Receptors, Muscarinic; 0/TNFSF13B protein, human |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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