What causes extraglandular involvement in the pathogenesis of Sjögren syndrome?

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

Extraglandular involvement in Sjögren syndrome manifests in part as hypergammaglobulinemia and the production of multiple autoantibodies, especially ANA and RF. This may be due to polyclonal B-cell activation, but the cause of this expanded activation is not known.

Involvement of other organs and tissues may result from effects of these antibodies, immune complexes, or lymphocytic infiltration and occurs in one third of patients with Sjögren syndrome. Prolonged hyperstimulation of B cells may lead to disturbances in their differentiation and maturation and may account for the greatly increased incidence of lymphoma in these patients. [25]


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!