Document Detail


Pathogenesis of Sjögren's syndrome.
MedLine Citation:
PMID:  19568172     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Nikolay P Nikolov; Gabor G Illei
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Intramural; Review    
Journal Detail:
Title:  Current opinion in rheumatology     Volume:  21     ISSN:  1531-6963     ISO Abbreviation:  Curr Opin Rheumatol     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-13     Completed Date:  2009-11-04     Revised Date:  2012-03-08    
Medline Journal Info:
Nlm Unique ID:  9000851     Medline TA:  Curr Opin Rheumatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  465-70     Citation Subset:  IM    
Affiliation:
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.
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MeSH Terms
Descriptor/Qualifier:
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:
Z01 DE000704-06/DE/NIDCR NIH HHS; Z01 DE000704-07/DE/NIDCR NIH HHS; Z99 DE999999/DE/NIDCR NIH HHS
Chemical
Reg. No./Substance:
0/Autoantibodies; 0/B-Cell Activating Factor; 0/Interferon-alpha; 0/Receptors, Muscarinic; 0/TNFSF13B protein, human
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