Pathogenesis of sjögren’s

The pathogenesis of primary Sjögren’s (pSS) remains an area of intense research. A triggering factor, either endogenous, leading to autoantigen release, or an external factor such as a virus, is thought to trigger inflammation of the ductal epithelial cells (autoimmune epitheliitis) and chronic focal inflammation of exocrine glands by inflammatory cells with a consequential reduction in glandular function. There is also a strong genetic link between HLA-DR3 and high levels of anti-Ro/La antibodies. More recently, gene association studies have confirmed this along with associations with genes involved in B-cell activation, type-I interferons, IL-12 and the innate immune system. Other immunological studies have also identified mechanisms of B and T-cell activation, activation of the type-I interferon pathway and a potential role for epigenetics in the pathogenesis of primary SS.