Sjogren syndrome

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 antinuclear antibodies, 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 Sep;9(9):544-56. 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 Mar;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 Nov;33(11):2227-34. http://www.ncbi.nlm.nih.gov/pubmed/17086607?tool=bestpractice.com [4]Steinberg AD, Talal N. The coexistence of Sjogren's syndrome and systemic lupus erythematosus. Ann Intern Med. 1971 Jan;74(1):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 May;66(5):733-6. 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 Jan;32(1):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 Jul;57(1):78-85. http://www.ncbi.nlm.nih.gov/pubmed/4134767?tool=bestpractice.com