Rheumatology Advance Access originally published online on June 14, 2005
Rheumatology 2005 44(11):1354-1367; doi:10.1093/rheumatology/keh714
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
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Primary Sjögren's syndrome: current and emergent aetiopathogenic concepts
Department of Autoimmune Diseases, IDIBAPS (Institut d'Investigacions Biomèdiques August Pi i Sunyer), Hospital Clinic, Barcelona, Spain.
Correspondence to: M. Ramos-Casals, Department of Autoimmune Diseases, Hospital Clínic, C/Villarroel, 170, 08036-Barcelona, Spain. E-mail: mramos@clinic.ub.es
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Introduction |
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Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects the exocrine glands and usually presents as persistent dryness of the mouth and eyes due to functional impairment of the salivary and lacrimal glands [1, 2]. The common histolopathological feature of all organs affected is a potentially progressive lymphocytic infiltration. Salivary glands are the most studied organs because they are affected in almost all patients and are easily accessible. Microscopic examination of the salivary glands reveals a benign lymphoepithelial lesion, characterized by lymphocytic replacement of the salivary epithelium and the presence of epimyoepithelial islands composed of keratin-containing epithelial cells. The predominant cells in the minor labial salivary gland infiltrates are T cells, with a bias towards CD4+ cells rather than CD8+ suppressor cells (CD4/CD8 ratio of 3:1–5:1). B cells constitute approximately 20% of the total infiltrating population, while natural killer (NK) cells are observed less often
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Autoimmune aetiopathogenesis |
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Genetic background
Polymorphisms of cytokine genes
Other polymorphic genes
Altered immune recognition
Autoantigens
Viruses
Abnormal immune responses
T-cell dysfunction
B-cell hyperreactivity
Cytokines and chemokines
Apoptotic mechanisms
Fas/FasL system
TNF
/TNFR-1 systemTRAIL/TRAIL-R system
PD-1/PD-1L system
p53/p21 system
Bcl-2/Bax system
Caspase system
Perforin/granzyme B system
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Emerging aetiopathogenic concepts |
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New candidate antigens
Hormonal factors
Autonomic dysfunction
Altered epithelial repair
Proteolytic mechanisms
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Conclusion |
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Connotea 
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