Underlying pathogenesis of primary Sjogrens Syndrome

What is the underlying pathogenesis of primary Sjogrens Syndrome?

The manifestations of SS result from a lymphocytic infiltration of glandular and nonglandular organs. Over 90% of the infiltrating cells are either CD4+ T lymphocytes with memory phenotype (70%) or B lymphocytes (20%). The remaining 10% include plasma cells, CD8+ T lymphocytes, T regulatory cells, natural killer cells, and dendritic cells. Analysis of T-cell cytokines from biopsies suggests that this is a predominantly Th1- and Th17-driven response.

There are two models of pathogenesis in SS. One postulates that an autoimmune attack on acinar epithelium results in cytotoxic cell death and apoptosis. The other model includes antibodies against the muscarinic receptor type 3, accelerated breakdown of acetylcholine by cytokines, and altered expression of aquaporin channels. Both models likely play a role in gland hypofunction.

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