Case 17
History:
45 year old female with bilateral parotid enlargement with palpable abnormalities
Findings:
MRI examination showing bilateral parotid gland enlargement with bilateral cysts and enhancing solid lesions.
Diagnosis:
Sjogren's syndrome with lymphoma
Discussion:
Sjogren’s syndrome with lymphoma is probably an autoimmune multisystem disorder. Characteristics include dryness of mucous membranes affecting salivary and lacrimal glands, pharyngeal mucosa, tracheobronchial tree, reticuloendothelial system,and joints. The syndrome affects adults between 35-70 years old. M:F=1:9. RF + 95%. ANA + IN 80%.
Diagnostic criteria for Sjogren’s Syndrome typically includes: Schirmer test, Keratoconjunctitis, Xerostomia, Extensive lymphocytic infiltrate on minor salivary gland biopsy, Laboratory evidence of a systemic autoimmune disorder.
Ultrasound shows enlarged salivary glands with multiple scattered cysts and increased vascularity on doppler. MRI shows inhmogeneous honeycomblike internal septation pattern, bilateral cysts. B Cell-Lymphomatous transformation, typically low grade, occurs in approximately 5 to 10 percent of patients.
SICCA SYNDROME (Mikulicz disease) : xerophthalmia and xerostomia.
References:
- Talal N. Sjogren's syndrome In: Schumacher HR, Jr., ed. Primer on the Rheumatic Diseases. 9th ed. Atlanta: Arthritis Foundation, 1988:136-8.
- Mariette X. Gougerot-Sjogren syndrome. Risk of lymphoma. Presse Med 1999;28:1214-18.
- Voulgarelis M, Moutsopoulos HM. Lymphoproliferation in autoimmunity and Siogren's syndrome. Curr Rheumatol Rep 2003;5:317-23.
