| Sjogren's Syndrome
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Etiology
•Unknown
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Pathogenesis
• Lymphocytic infiltration of salivary and lacrimal glands followed by destruction of normal tissue and fibrosis
• CD4+ T cells in infiltrate; autoantibodies in circulation including rheumatoid factor (75%), ANA (>50%), and anti-ribonucleoprotein antibodies including SS-A(Ro) and SS-B (La) which are most common,
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Epidemiology
• Most are women between 40 and 60
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General Gross Description
• Rarely see glands but fleshy and enlarged initially then atrophic and fatty
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General Microscopic Description
• Lymphocytic infiltrates in periductal and perivascular regions early
• Coalesce to form sheets of lymphocytes with follicle formation
• Eventual atrophy of duct structures, fatty replacement and fibrosis
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Clinical Correlation
• Present with dry eyes and dry mouth (keratoconjunctivitis and xerostomia)
• Isolated disorder is sicca syndrome; secondary to variety of autoimmune diseases esp rheumatoid arthritis
• Ulceration of cornea with inflammation due to lack of tears
• Dry, fissured mouth and ulcerated nose which may perforate
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References
• Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 208-210.
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| Sjogren's Syndrome
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| Synopsis by: Melinda Sanders M.D. (T55100M72240)[332]
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