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Sjgren syndrome (gougerot-sjgren syndrome; secretoinhibitor syndrome; sicca syndrome) 1


Sjgren Syndrome (Gougerot-Sjgren Syndrome; Secretoinhibitor Syndrome; Sicca Syndrome) 1

General: Etiology unknown; autosomal recessive; occurs in women over age 40 years; failure of the lacrimal and conjunctiv 343f59d al glands to maintain adequate secretion; similarities exist with Mikulicz syndrome (see Mikulicz-Radecki Syndrome); insidious onset; associated with collagen disorders; Epstein-Barr virus infection.

Ocular: Blepharoconjunctivitis; tears show no lysozyme; keratoconjunctivitis sicca; superficial corneal ulcers; thready, tenacious, yellow-white discharge of the conjunctiva; hypertrophy of lacrimal gland; decreased tear secretion with cellular and mucous debris in tear film; cicatrization of cornea and conjunctiva.

Clinical: Dryness of mouth and other mucous membranes; enlarged salivary glands; dysphagia; painless swelling of joints; polyarthritis; dental cavities; vaginitis; laryngitis; rhinitis sicca; hepatomegaly; focal myositis; alopecia; splenomegaly.

Fischbach M, et al. Immune complexes in Sjogren's syndrome. Arthritis Rheum 1980; 23:791-795.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Friedalaender MH. Ocular manifestations of Sjogren's syndrome: keratoconjunctivitis sicca. Rheum Dis Clin North Am 1992; 18:591-608.

Gudas PP, et al. Corneal perforations in Sjogren's syndrome. Arch Ophthalmol 1973; 90:470.

Miyasaka N, et al. Possible involvement of Epstein-Barr virus in the pathogenesis of Sjogren's syndrome. Clin Immunol Immunopathol 1994; 72:166-l70.

Sjogren H. Zur Kenntnis der Keratoconjunctivitis Sicca. Acta Ophthalmol 1933[Suppl 2].

Williamson J, et al. management of the dry eye in Sjogren's syndrome. Br J Ophthalmol 1974; 58:798-805.

Wu AJ, Fox PC. Sjogren's syndrome. Semin Dermatol 1994; 13:138-l43.




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