Sjögren's Syndrome

Abstract

Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterised 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 is also associated with an increased risk of B‐cell lymphoma development. Current treatments are mainly symptomatic.

Key Concepts

  • Development and validation of proposed consensus criteria for Sjögren's syndrome jointly by the ACR‐EULAR Sjögren's Syndrome Classification Criteria Working Group are shortly to be finalised.
  • Validated disease activity and damage indexes have been developed.
  • Ultrasonography of major salivary glands is a coming investigative procedure.
  • Treatment with biological agents is used in selected cases with systemic manifestations.
  • The prevalence of Sjögren's syndrome is about the same as for systemic lupus erythematosus.
  • Ectopic germinal centres and high focus score have been proposed at predictors for lymphoma development in addition to previously verified biomarkers.
  • Interferon signature is an important pathophysiological finding.
  • Saliva is a promising source of biologically relevant biomarker signatures.
  • Recent GWAS have identified novel candidate genes.

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. (a) American‐European Consensus Group Criteria. For details see Vitali et al. (). (b) Comparison of the AECC and the ACR criteria.
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 organised 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. ().
Figure 5. Schematic representation of current views of the aetiopathogenesis of Sjögren's syndrome. Adapted from Price and Venables (1995) © 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.

Eriksson P and Jonsson R (1999) The 100‐year anniversary of Henrik Sjögren (proceedings + abstracts). Hygiea 108 (suppl 1): 1–114.

Isenberg DA and Horsfall AC (eds) (1994) Autoimmune Diseases: Focus on Sjögren's Syndrome. Oxford: Bios Scientific Publishers.

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.

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.

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.

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.

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Jonsson, Roland, and Brun, Johan G(Sep 2015) Sjögren's Syndrome. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002149.pub3]