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Sjögren's Syndrome

  1. Roland Jonsson1,
  2. Johan G Brun2

Published Online: 15 FEB 2010

DOI: 10.1002/9780470015902.a0002149.pub2

eLS

eLS

How to Cite

Jonsson, R. and Brun, J. G. 2010. Sjögren's Syndrome. eLS. .

Author Information

  1. 1

    University of Bergen, Broegelmann Research Laboratory, The Gade Institute, Bergen, Norway

  2. 2

    Haukeland University Hospital, Department of Rheumatology, Bergen, Norway

Publication History

  1. Published Online: 15 FEB 2010

This is not the most recent version of the article. View current version (14 SEP 2015)

Abstract

Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterized by mononuclear cell infiltration of exocrine glands, notably the lacrimal and salivary glands (autoimmune exocrinopathy). These lymphoid infiltrations lead to dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia) and frequently, dryness of the nose, throat, vagina and skin. Sjögren's syndrome is associated with the production of autoantibodies because B-cell activation is a consistent immunoregulatory abnormality. The spectrum of the disease extends from an organ-specific autoimmune disorder to a systemic process (musculoskeletal, pulmonary, gastric, haematologic, dermatologic, renal and nervous system involvement). Sjögren's syndrome may occur alone (primary) or in association with almost any of the autoimmune rheumatic diseases (secondary), the most frequent being rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Sjögren's syndrome also is associated with an increased risk of B-cell lymphoma development. Current treatments are mainly symptomatic.

Keywords:

  • autoantibodies;
  • lacrimal glands;
  • non-Hodgkin lymphoma;
  • Salivary glands;
  • sicca symptoms;
  • Sjögren's syndrome