Sjögren's syndrome pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farbod Zahedi Tajrishi, M.D.

Overview

ُSjögren's syndrome is a chronic auto-immune disorder that can affect several organ systems. Both genetic and immune factors contribute to the pathogenesis of the disease. In the most common presentation of the disease, lymphocytes infiltrate the lacrimal and salivary glands and impair their function, hence causing the main characteristic symptoms- dry mouth (keratoconjunctivitis sicca) and eyes (xerostomia). CD4+ T-cells are predominant in mild and moderate salivary gland infiltrations, while B cells play the major role in severe lesions [1].

Pathophysiology

The pathogenesis of Sjögren's syndrome can be linked to both genetic and nongenetic components [2].

  • Genetic components:

Although multiple genes are found to be associated with ُSjögren's syndrome, evidence suggests genes that encode Major Histocompatibility Complex (MHC), particularly HLA genes, play an important role in the disease.

  • Environmental components:
    • Several studies have indicated an association between Sjögren's syndrome and some viral infections.
  • Immune-mediated components:

References

  1. Christodoulou MI, Kapsogeorgou EK, Moutsopoulos HM (2010). "Characteristics of the minor salivary gland infiltrates in Sjögren's syndrome". J Autoimmun. 34 (4): 400–7. doi:10.1016/j.jaut.2009.10.004. PMID 19889514.
  2. Mavragani CP, Nezos A, Moutsopoulos HM (2013). "New advances in the classification, pathogenesis and treatment of Sjogren's syndrome". Curr Opin Rheumatol. 25 (5): 623–9. doi:10.1097/BOR.0b013e328363eaa5. PMID 23846338.

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