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Sjögren’s Syndrome

  • Chapter
Book cover Primer on the Rheumatic Diseases

Abstract

  • Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease with early and gradually progressive lacrimal and salivary dysfunction.

  • Secondary SS occurs in association with other autoimmune disorders, the most common of which is rheumatoid arthritis.

  • Minor salivary glands and lacrimal glands in SS exhibit a particular pattern of periductal lymphocytic infiltration known as focal lymphocytic sialadenitis.

  • About 90% of patients with SS are women.

  • Sjögren’s syndrome is very common, with a community prevalence of pSS ranging from 0.1% to 0.6% of all individuals.

  • The major eye problem in SS is keratoconjunctivitis sicca, leading to xerophthalmia. The principal oral manifestation of SS is decreased salivary gland production, leading to xerostomia and a predilection for dental caries.

  • Extraglandular manifestations of SS include arthralgias, thyroiditis, renal involvement (leading to renal tubular acidosis), peripheral neuropathy, cutaneous vasculitis, and lymphoma.

  • The risk of lymphoma in pSS is approximately 5%.

  • Most patients with SS develop increased circulating polyclonal immunoglobulins and autoantibodies. These autoantibodies include two fairly specific antibodies directed against the Ro (SS-A) and La (SS-B) antigens.

  • ■ Anti-Ro and -La antibodies may be associated with fetal heart block during the pregnancies of women with SS.

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Author information

Authors and Affiliations

  1. Schools of Dentistry and Medicine, University of California, San Francisco, San Francisco, CA, USA

    Troy Daniels DDS, MS (Professor)

Authors
  1. Troy Daniels DDS, MS
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Editors and Affiliations

  1. Arthritis Foundation, Atlanta, GA, USA

    John H. Klippel MD (President and CEO) & Patience H. White MD, MA (Chief Public Health Officer) (President and CEO) &  (Chief Public Health Officer)

  2. Massachusetts General Hospital, Deputy Editor for Rheumatology UpToDate, Boston, MA, USA

    John H. Stone MD, MPH (Associate Physician) (Associate Physician)

  3. Division of Rheumatology & Women’s Health, University of Kentucky, Lexington, KY, USA

    Leslie J. Crofford MD (Gloria W. Singletary Professor of Internal Medicine, Chief) (Gloria W. Singletary Professor of Internal Medicine, Chief)

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© 2008 Springer Science+Business Media, LLC.

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Daniels, T. (2008). Sjögren’s Syndrome. In: Klippel, J.H., Stone, J.H., Crofford, L.J., White, P.H. (eds) Primer on the Rheumatic Diseases. Springer, New York, NY. https://doi.org/10.1007/978-0-387-68566-3_50

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  • DOI: https://doi.org/10.1007/978-0-387-68566-3_50

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-35664-8

  • Online ISBN: 978-0-387-68566-3

  • eBook Packages: MedicineMedicine (R0)

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