The cause of SS is unknown, but several stages of its pathogenesis are being elucidated. The initial event may be activation of ductal epithelial cells by an unknown antigen. Expression of co-stimulatory molecules [i.e., HLA-DR, CD80, CD86, intracellular adhesion molecule (ICAM)] by ductal epithelium highlights an unexpected role in the regulation of the immune response. Affected organs exhibit periductal lymphocytic infiltrates composed initially of CD4+ T-cells, which can subsequently attract B-cells. The T-cells release cytokines
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