Etiology of Sjögren's Syndrome
SS is a systemic autoimmune disease resulting from the interplay of environmental and genetic factors.[16] This complex interaction leads to a local lymphocytic infiltration of the lacrimal and salivary glands and the systemic production of autoantibodies. Rheumatoid factor (RF), antinuclear antibodies (ANA), and antinucleoproteins (extractable nuclear antigens; SS-A [Ro] and SS-B [La]) are commonly associated with SS.[17,18] Although no specific agent has been identified, it has been suggested that SS may be triggered by an abnormal response to viral antigens or virally altered host antigens.[19] Cytomegalovirus, paramyxovirus, Epstein-Barr virus, and hepatitis C virus have received wide attention as possible etiologic agents. Similarly, recent studies suggest a relationship between SS and retroviruses.[20,21] It has been hypothesized that these supposed etiologic agents could trigger an immune dysregulation in a genetically susceptible individual, resulting in the development of SS. This hypothesis was supported in part by studies showing that particular histocompatibility antigens (HLA-B8, HLA-DR3, HLA-DRw52) occur with a higher frequency among patients with SS.[17,18]
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