Sjögren's Syndrome

Roland Jonsson, University of Bergen, Bergen, Norway
Johan G Brun, Haukeland University Hospital, Bergen, Norway

Published online: February 2010

DOI: 10.1002/9780470015902.a0002149.pub2

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

Figure 1. Proposed aetiopathogenic events before diagnosis of Sjögren's syndrome.
Figure 2. American-European Concensus Group Criteria. For details see Vitali et al. (2002).
Figure 3. Microscopical view of the chronic inflammation in minor salivary glands obtained from the inside of the lower lip of a patient with Sjögren's syndrome. (a) Multiple focal infiltrates. (b) High-power view of organized lymphoid tissue (ectopic germinal centres).
Figure 4. Microscopical view of local production of antigen-specific autoantibodies in minor salivary glands in Sjögren's syndrome. For further details see Tengnér et al. (1998).
Figure 5. Schematic representation of current views of the aetiopathogenesis of Sjögren's syndrome. Adapted from Price and Venables (1995), with permission from Elsevier.
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 Further reading
    Delaleu N, Jonsson MV and Jonsson R (2004) Disease mechanisms of Sjögren's syndrome. Drug Discovery Today 1: 329–336.
    other Eriksson P and Jonsson R (eds) (1999) The 100-year anniversary of Henrik Sjögren (proceedings + abstracts). Hygiea 108(suppl 1): 1–114.
    book Isenberg DA and Horsfall AC (eds) (1994) Autoimmune Diseases: Focus on Sjögren's Syndrome. Oxford: Bios Scientific Publishers.
    other Jonsson MV (2006) Ectopic Germinal Center Formation in Sjögren's Syndrome – Significance of Lymphoid Organization. PhD thesis, University of Bergen, Bergen, Norway. ISBN: 82-308-0176-2.
    book Jonsson R, Bowman S and Gordon TP (2005) "Sjögren's syndrome". In: Koopman WJ and Moreland LW (eds) Arthritis and Allied Conditions – A Textbook of Rheumatology, 15th edn, pp. 1681–1705. Philadelphia: Lippincott Williams & Wilkins.
    book Jonsson R and Brokstad K (2001) "Sjögren's syndrome". In: Austen KF, Frank MM, Atkinson JP and Cantor H (eds) Samter's Immunologic Diseases, 6th edn, "Chapter 40", pp. 495–504. Philadelphia: Lippincott Williams & Wilkins.
    book Keech CL, McCluskey J and Gordon TP (1996) "SS-B(La) autoantibodies". In: Peter JB and Schoenfeld Y (eds) Autoantibodies, pp. 789–797. Amsterdam: Elsevier.
    book Reichlin M and Scofield RH (1996) "SS-A(Ro) autoantibodies". In: Peter JB and Schoenfeld Y (eds) Autoantibodies, pp. 783–788. Amsterdam: Elsevier.
    book Talal N, Moutsopoulos HM and Kassan SS (eds) (1987) Sjögren's Syndrome: Clinical and Immunological Aspects. Berlin: Springer-Verlag.
    Workshop on Diagnostic Criteria for Sjögren's Syndrome (1989) I. Questionnaires for dry eye and dry mouth. II. Manual of methods and procedures. Clinical and Experimental Rheumatology 7: 212–219.

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Article Title: Sjögren's Syndrome
 
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Jonsson, Roland, and Brun, Johan G(Feb 2010) Sjögren's Syndrome. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002149.pub2]