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Primary Sjögren's Syndrome After Infectious Mononucleosis

Stephen C. Pflugfelder, MD; Thomas J. Roussel, MD; William W. Culbertson, MD
JAMA. 1987;257(8):1049-1050. doi:10.1001/jama.1987.03390080039017.
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To the Editor.—  Primary Sjögren's syndrome is an immune disorder consisting of keratoconjunctivitis sicca and dry mucosal surfaces without an associated connective-tissue disorder.1 This disease has characteristic autoantibodies, HLA association, and glandular histopathology.2-4 We have examined two patients with primary Sjögren's syndrome occurring after infectious mononucleosis.

Report of Cases.—Case 1.—  A 5-year-old girl had parotid gland swelling, fever, and pharyngitis. Her white blood cell count was 9200/mm3 (9.2×109/L), with 7% (0.07) atypical lymphocytes. Epstein-Barr virus (EBV) titers one month after onset were as follows: viral capsit antigen (VCA) IgM, 1:40; VCA IgG, 1:320; and Epstein-Barr virus— associated nuclear antigen, 1:128. The serum IgG level was seven times normal and circulating immune complexes were present. Over the next two months, she was noted to have no tears when she cried. A Schirmer's test revealed 3 mm of wetting; 50% of the interpalpebral zone stained


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