What is the pathogenesis of Sjögren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Answer

The etiology of Sjögren syndrome is not well understood. The presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules and the identification of inherited susceptibility markers suggest that environmental or endogenous antigens trigger a self-perpetuating inflammatory response in susceptible individuals. In addition, the continuing presence of active interferon pathways in Sjögren syndrome suggests ongoing activation of the innate immune system. [7, 8] Together, these findings suggest an ongoing interaction between the innate and acquired immune systems in Sjögren syndrome.


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