Chronic inflammatory and autoimmune disorder characterized by diminished lacrimal and salivary gland secretion (sicca complex).
May present with fatigue and dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).
Diagnosed by positive ANAs, anti-60 kD (SS-A) Ro and anti-La autoantibodies, decreased saliva and tear production, lymphocytic infiltration in labial salivary gland biopsy.
Treatment mainly symptomatic.
Sjogren syndrome is a systemic autoimmune disorder,
[1]
Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjogren's syndrome. Nat Rev Rheumatol. 2013;9:544-556.
http://www.ncbi.nlm.nih.gov/pubmed/23857130?tool=bestpractice.com
characterized by the presence of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) as a consequence of lymphocytic infiltration into the lacrimal and salivary glands. Additional symptoms can include dryness of skin, nose, throat, vagina; arthralgias and myalgias; peripheral neuropathies; pulmonary, thyroid, and renal disorders; and lymphoma.
[2]
Fox PC. Autoimmune diseases and Sjogren's syndrome, an autoimmune exocrinopathy. Ann N Y Acad Sci. 2007;1098:15-21.
http://www.ncbi.nlm.nih.gov/pubmed/17332090?tool=bestpractice.com
Sjogren syndrome can occur alone (primary Sjogren syndrome) or along with another autoimmune disease (secondary Sjogren syndrome) such as lupus, rheumatoid arthritis, and systemic sclerosis.
[3]
Anaya JM, Tobon GJ, Vega P, et al. Autoimmune disease aggregation in families with primary Sjogren's syndrome. J Rheumatol. 2006;33:2227-2234.
http://www.ncbi.nlm.nih.gov/pubmed/17086607?tool=bestpractice.com
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Steinberg AD, Talal N. The coexistence of Sjogren's syndrome and systemic lupus erythematosus. Ann Intern Med. 1971;74:55-61.
http://www.ncbi.nlm.nih.gov/pubmed/5312811?tool=bestpractice.com
[5]
Moutsopoulos HM, Webber BL, Vlagopoulos TP, et al. Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. Am J Med. 1979;66:733-736.
http://www.ncbi.nlm.nih.gov/pubmed/443249?tool=bestpractice.com
[6]
Foster H, Fay A, Kelly C, et al. Thyroid disease and other autoimmune phenomena in a family study of primary Sjogren's syndrome. Br J Rheumatol. 1993;32:36-40.
http://www.ncbi.nlm.nih.gov/pubmed/8422556?tool=bestpractice.com
[7]
Alarcon-Segovia D, Ibanez G, Hernandez-Ortiz J, et al. Sjogren's syndrome in progressive systemic sclerosis (scleroderma). Am J Med. 1974;57:78-85.
http://www.ncbi.nlm.nih.gov/pubmed/4134767?tool=bestpractice.com