Definition
Sjögren syndrome is a chronic, progressive autoimmune disorder that primarily affects the exocrine glands. The classic signs and symptoms include enlargement of the parotid and lacrimal glands, with mucosal dryness manifested by dry mouth (xerostomia) and dry eyes (xerophthalmia) (keratoconjunctivitis sicca). Lymphocytic infiltrates replace the functional epithelium, which results in decreased exocrine secretions (exocrinopathy).
Numerous features suggest an immunologic etiology. Patients demonstrate hypergammaglobulinemia and have several types of autoantibodies, such as the rheumatoid factor (RF), antinuclear antibodies (ANA, i.e., autoantibody against a nuclear substance), Ro/SSA and/or La/SSB, and antibodies to the salivary duct epithelium.
Characteristics
Sjögren syndrome is named after the Swedish ophthalmologist Henrik Sjögren....
References
Carbone A, Glogghini A (2000) Pathological features of lymphoid proliferations of the salivary glands: lymphoepithelial sialadenitis versus low-grade B-cell lymphoma of the malt type. Ann Otol Rhinol Laryngol 109:1170–1175
Masaki Y, Sugai S (2003) Lymphoproliferative disorders in Sjögren syndrome. Autoimmun Rev 3:175–182
Tsay GJ, Hsu TC (2006) Anti-CENP-H in patients with Sjögren syndrome. Rheumatol Int 26:298–303
Tsay GJ, Wang C-L (1996) SSA/Ro antibodies in Chinese patients with systemic lupus erythematosus and Sjögren syndrome. J Formos Med Assoc 95:905–910
Vitali C, Bombardieri S, Moutsopoulos HM et al (1993) Preliminary criteria for the classification of Sjögren syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum 36:340–347
See Also (part 1)
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(2012) Antigen. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 209. doi:10.1007/978-3-642-16483-5_319
(2012) Anti-Ro/SSA and Anti-La/SSB antibodies. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 217. doi:10.1007/978-3-642-16483-5_308
(2012) Autoantigens. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 311. doi:10.1007/978-3-642-16483-5_467
(2012) Autoimmune diseases. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 311. doi:10.1007/978-3-642-16483-5_473
(2012) Autoimmune response. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 312. doi:10.1007/978-3-642-16483-5_476
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(2012) CD5 B cells. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 702. doi:10.1007/978-3-642-16483-5_916
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See Also (part 2)
(2012) Immunoglobulin. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1819. doi:10.1007/978-3-642-16483-5_2990
(2012) Immunoglobulins. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 1819–1820. doi:10.1007/978-3-642-16483-5_2994
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(2012) Interleukin-1. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1892. doi:10.1007/978-3-642-16483-5_3095
(2012) Interleukin-2. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1892. doi:10.1007/978-3-642-16483-5_3097
(2012) Leukocyte functional antigens. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2027. doi:10.1007/978-3-642-16483-5_3327
(2012) Lymph. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2116. doi:10.1007/978-3-642-16483-5_3442
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(2012) Major histocompatibility complex. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2137. doi:10.1007/978-3-642-16483-5_3500
(2012) Molecular mimicry. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2358. doi:10.1007/978-3-642-16483-5_3815
(2012) Monoclonal antibody. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2367. doi:10.1007/978-3-642-16483-5_6842
(2012) P53. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2747. doi:10.1007/978-3-642-16483-5_4331
(2012) Perforin. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2814. doi:10.1007/978-3-642-16483-5_4443
(2012) Ran. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3164. doi:10.1007/978-3-642-16483-5_4943
(2012) Rheumatoid arthritis. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3302. doi:10.1007/978-3-642-16483-5_5097
(2012) Ribavirin. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3308. doi:10.1007/978-3-642-16483-5_5101
(2012) RT-PCR. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3322. doi:10.1007/978-3-642-16483-5_5129
(2012) Seckel syndrome. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3342. doi:10.1007/978-3-642-16483-5_5190
(2012) Systemic lupus erythematosus. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3598. doi:10.1007/978-3-642-16483-5_5639
(2012) T Cell. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3599. doi:10.1007/978-3-642-16483-5_5645
(2012) T lymphocyte. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3600. doi:10.1007/978-3-642-16483-5_5654
(2012) Target cells. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3607. doi:10.1007/978-3-642-16483-5_5671
(2012) TCRα and TCRβ. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3629. doi:10.1007/978-3-642-16483-5_5708
(2012) Tumor necrosis factor-α. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3800. doi:10.1007/978-3-642-16483-5_6041
(2012) Viruses. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3924. doi:10.1007/978-3-642-16483-5_6203
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Chou, PL., Tsay, G.J. (2014). Sjögren Syndrome. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_5333-2
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