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Sjögren's Syndrome

  • Chapter
A Clinician's Pearls and Myths in Rheumatology

Abstract

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.

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Authors and Affiliations

  1. Servei de Malalties Autoimmunes, Hospital Clínic, C/Villarroel, Barcelona, 170, 08036, Spain

    Manuel Ramos-Casals

  2. University of California at San Franscisco School of Medicine and Dentistry, Orofacial Sciences, Parnassus Avenue, 0422, 521, San Francisco, CA, 94143-2206

    Troy E. Daniels

  3. Rheumatology Clinic, Scripps Memorial Hospital, 9850 Genesee Avenue, Suite 910, La Jolla, CA, 92037, USA

    Robert I. Fox

  4. University of Californa-San Francisco Department of Ophthalmology, San Francisco, California

    John P. Whitcher

  5. National University of Athens Medical School Department of Pathophysiology, 75 Mikras Asias Str, Athens, 115 27, Greece

    George E. Fragoulis, Fotini N. Skopouli & Haralampos M. Moutsopoulos

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  1. Manuel Ramos-Casals
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  2. Troy E. Daniels
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  3. Robert I. Fox
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  5. George E. Fragoulis
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  6. Fotini N. Skopouli
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  7. Haralampos M. Moutsopoulos
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  1. Clinical Rheumatology, Massachusetts General Hospital, 55 Fruit Street / Yawkey 2, Boston, MA, 02114

    John H. Stone (Director) (Director)

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Ramos-Casals, M. et al. (2009). Sjögren's Syndrome. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_12

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