Document Detail

The geoepidemiology of Sj?gren's syndrome.
MedLine Citation:
PMID:  19903539     Owner:  NLM     Status:  MEDLINE    
Sjogren's syndrome (SS) is a slowly progressing autoimmune disease, affecting predominantly middle-aged women, with a female to male ratio reaching 9:1. It is characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in reduced secretory functions and oral and ocular dryness. The syndrome can present alone as primary SS (pSS) or in the context of underlying connective tissue disease as secondary SS (sSS). While the pathogenesis of the disease remains elusive, environmental, genetic and hormonal contributors seem to be involved. Over the last years, compelling evidence has suggested a pivotal role of the epithelium in orchestrating the immune response in the histopathological lesion of Sjogren's syndrome and the term "autoimmune epithelitis" has been proposed as an etiological term. Although the clinical manifestations of pSS patients are mainly those of an autoimmune exocrinopathy, almost half of patients develop extraglandular disease, which may be manifested either by epithelial lymphocytic invasion of lung, liver, or kidney (resulting in interstitial nephritis) or by skin vasculitis, peripheral neuropathy, glomerulonephritis, and low C4 levels. The latter reflect immune-complex mediated disease and confer increased risk for lymphoma development.
Clio P Mavragani; Haralampos M Moutsopoulos
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Publication Detail:
Type:  Journal Article; Review     Date:  2009-11-10
Journal Detail:
Title:  Autoimmunity reviews     Volume:  9     ISSN:  1873-0183     ISO Abbreviation:  Autoimmun Rev     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-03-09     Completed Date:  2010-06-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101128967     Medline TA:  Autoimmun Rev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  A305-10     Citation Subset:  IM    
Copyright Information:
2009 Elsevier B.V. All rights reserved.
Department of Experimental Physiology, University of Athens, School of Medicine, Athens, Greece.
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MeSH Terms
Autoimmune Diseases / epidemiology*,  immunology*,  pathology
Lacrimal Apparatus / pathology
Salivary Gland Diseases / pathology
Salivary Glands / pathology
Sjogren's Syndrome / epidemiology*,  immunology*,  pathology

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