Sjögren's Syndrome

  • Manuel Ramos-Casals
  • Troy E. Daniels
  • Robert I. Fox
  • John P. Whitcher
  • George E. Fragoulis
  • Fotini N. Skopouli
  • Haralampos M. Moutsopoulos


Primary Sjögren's syndrome (SjS) is a systemic autoimmune disease that is associated with early and gradually progressive lacrimal and salivary dysfunction. Secondary SjS occurs in association with other autoimmune disorders, the most common of which is rheumatoid arthritis. About 90% of patients with SjS are women. Minor salivary glands and lacrimal glands in SjS exhibit a particular pattern of periductal focal lymphocytic infiltration known as focal lymphocytic sialadenitis. Primary SjS has a community prevalence that ranges from 0.1 to 0.6%. The major eye problem in SjS is keratoconjunctivitis sicca, which leads to xerophthalmia. The principal oral manifestation of SjS is decreased salivary gland production, leading to xerostomia and a predilection for dental caries. Extraglandular manifestations of SjS include arthral-gias, thyroiditis, renal involvement (leading to renal tubular acidosis (RTA)), peripheral neuropathy, cutaneous vasculitis, and lymphoma. The risk of lymphoma in SjS is approximately 5%. Most patients with SjS 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.


Salivary Gland Mixed Cryoglobulinemia Burning Mouth Syndrome Keratoconjunctivitis Sicca Gland Enlargement 
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.


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Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • Manuel Ramos-Casals
    • 1
  • Troy E. Daniels
    • 2
  • Robert I. Fox
    • 3
  • John P. Whitcher
    • 4
  • George E. Fragoulis
    • 5
  • Fotini N. Skopouli
    • 5
  • Haralampos M. Moutsopoulos
    • 5
  1. 1.Servei de Malalties Autoimmunes, Hospital ClínicC/VillarroelSpain
  2. 2.University of California at San Franscisco School of Medicine and Dentistry, Orofacial SciencesSan Francisco
  3. 3.Rheumatology Clinic, Scripps Memorial HospitalLa JollaUSA
  4. 4.University of Californa-San Francisco Department of OphthalmologySan FranciscoCalifornia
  5. 5.National University of Athens Medical School Department of PathophysiologyAthensGreece

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