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Sjögren’s Syndrome

  • Troy Daniels

Abstract

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease with gradually progressive lacrimal and salivary dysfunction, which can be symptomatic or asymptomatic and include a variety of extraglandular conditions. Secondary Sjögren’s syndrome (sSS) occurs when lacrimal and salivary dysfunction develop in patients with another autoimmune connective tissue disease (ACTD), most commonly rheumatoid arthritis. Because of the persistence and progression of secretory dysfunction, patients with either form of SS often experience significant misery. The community prevalence of pSS, using current diagnostic criteria, ranges from 0.1% to 0.6%, depending on the study design. Affected organs in patients with SS exhibit a particular pattern of chronic inflammation that is gradually progressive and uncommonly undergoes transformation to lymphoma. Patients with SS produce a variety of circulating autoantibodies, but no combination of these has yet been established as a satisfactory classification criterion for pSS, which requires multisystem diagnostic tests. Treatment of SS requires effective management of both ocular and oral secretory dysfunction, the prevention or treatment of disease sequelae, and therapy for any extraglandular conditions that occur. There is currently no single treatment that addresses all of the diverse manifestations of this disease.

Keywords

Primary Biliary Cirrhosis Minor Salivary Gland Congenital Heart Block Keratoconjunctivitis Sicca Saliva Substitute 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Bowman S, Ibrahim G, Holmes G, Hamburger J, Ainsworth J. Estimating the prevalence among Caucasian women of primary Sjögren’s syndrome in two general practices in Birmingham, UK. Scand J Rheumatol 2004;33:39–43.CrossRefPubMedGoogle Scholar
  2. 2.
    Gottenberg J, Busson M, Loiseau P, et al. In primary Sjögren’s syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response. Arthritis Rheumatol 2003;48:2240–2245.CrossRefGoogle Scholar
  3. 3.
    Triantafyllopoulou A, Tapinos N, Moutsopoulos H. Evidence for Coxsackievirus infection in primary Sjögren’s syndrome. Arthritis Rheumatol 2004;50:2897–2902.CrossRefGoogle Scholar
  4. 4.
    Ramos-Casals M, Loustaud-Ratti V, DeVita S, et al. Sjögren syndrome associated with hepatitis C virus. A multicenter analysis of 137 cases. Medicine 2005;84:81–89.CrossRefPubMedGoogle Scholar
  5. 5.
    Daniels T, Whitcher J. Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjögren’s syndrome. Arthritis Rheumatol 1994;37:869–877.CrossRefGoogle Scholar
  6. 6.
    Larsson C, Bredberg A, Henriksson G, Manthorpe R, Sallmyr A. Immunohistochemistry of the B-cell component in lower lip salivary glands of Sjögren’s syndrome and healthy subjects. Scand J Immunol 2005;61:98–107.CrossRefPubMedGoogle Scholar
  7. 7.
    Prochorec-Sobieszek M, Wagner T, Loukas M, Chwaliska-Sadowska H, Olesiska M. Histopathological and immunohistochemical analysis of lymphoid follicles in labial salivary glands in primary and secondary Sjögren’s syndrome. Med Sci Monit 2004;10:BR115–BR21.PubMedGoogle Scholar
  8. 8.
    Mitsias D, Tzioufas A, Veiopoulou C, et al. The Th1/Th2 cytokine balance changes with the progress of the immunopathological lesion of Sjögren’s syndrome. Clin Exp Immunol 2002;128:562–568.CrossRefPubMedGoogle Scholar
  9. 9.
    Mariette X, Roux S, Zhang J, et al. The level of BlyS (BAFF) correlates with the titer of autoantibodies in human Sjögren’s syndrome. Ann Rheum Dis 2003;62:168–171.CrossRefPubMedGoogle Scholar
  10. 10.
    Li J, Ha Y, Ku N, et al. Inhibitory effects of autoantibodies on the muscarinic receptors in Sjögren’s syndrome. Lab Invest 2004;84:1430–1438.CrossRefPubMedGoogle Scholar
  11. 11.
    Whitcher J, Gritz D, Daniels T. The dry eye: a diagnostic dilemma. Int Ophthalmal Clin 1998;38:23–37.CrossRefGoogle Scholar
  12. 12.
    Daniels T. Labial salivary gland biopsy in Sjögren’s syndrome. Assessment as a diagnostic criterion in 362 cases. Arthritis Rheumatol 1984;27:147–156.CrossRefGoogle Scholar
  13. 13.
    Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren’s syndrome. Clinical and immunologic disease patterns in a cohort of 400 patients. Medicine 2002;81:270–280.CrossRefPubMedGoogle Scholar
  14. 14.
    Matsumoto T, Morizane T, Aoki Y, et al. Autoimmune hepatitis in primary Sjögren’s syndrome: pathological study of the livers and labial salivary glands in 17 patients. Pathol Int 2005;55:70–76.CrossRefPubMedGoogle Scholar
  15. 15.
    Ramos-Casals M, Anaya JM, Garcia-Carrasco M, et al. Cutaneous vasculitis in primary Sjögren’s syndrome. Classification and clinical significance of 52 patients. Medicine 2004;83:96–106.CrossRefPubMedGoogle Scholar
  16. 16.
    Zintzaras E, Voulgarelis M, Moutsopoulos H. The risk of lymphoma development in autoimmune diseases. Arch Intern Med 2005;165:2337–2344.CrossRefPubMedGoogle Scholar
  17. 17.
    Ramos-Casals M, Font J, Garcia-Carrasco M, et al. Primary Sjögren’s syndrome. Hematologic pattern of disease expression. Medicine 2002;81:281–292.CrossRefPubMedGoogle Scholar
  18. 18.
    Ramos-Casals M, Brito-Zern P, Yage J, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjögren’s syndrome. Rheumatology 2005;44:89–94.CrossRefPubMedGoogle Scholar
  19. 19.
    Sjögren’s Syndrome Foundation. And the survey says … The Moisture Seekers 2006;24:1–3.Google Scholar
  20. 20.
    Vitali C, Bombardieri S, Moutsopoulos H, et al. Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheumatol 1993;36:340–347.CrossRefGoogle Scholar
  21. 21.
    Fox R, Robinson C, Curd J, Kozin F, Howell F. Sjögren’s syndrome. Proposed criteria for classification. Arthritis Rheumatol 1986;29:577–585.CrossRefGoogle Scholar
  22. 22.
    Vitali C, Bombardieri, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:544–558.CrossRefGoogle Scholar
  23. 23.
    Dawson L, Caulfield V, Stanbury J, Field A, Christmas S, Smith P. Hydroxychloroquine therapy in patients with primary Sjogren’s syndrome may improve salivary gland hypofunction by inhibition of glandular cholinesterase. Rheumatology 2005;44:449–455.CrossRefPubMedGoogle Scholar
  24. 24.
    Theander E, Manthorpe R, Jacobsson TH. Mortality and causes of death in primary Sjögren’s syndrome. Arthritis Rheum 2004;50:1262–1269.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Troy Daniels
    • 1
  1. 1.Schools of Dentistry and Medicine, University of California, San FranciscoSan FranciscoUSA

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