Sjogren Syndrome Flashcards
What is Sjogren syndrome?
A rare autoimmune exocrinopathy that causes a combination of symptoms/signs; dry eyes and dry mouth are the most notable.
Name the diagnostic criteria for Sjogren syndrome.
Inflamed and dry eyes (keratoconjunctivitis sicca); dry mouth (xerostomia); lymphocytic infiltrate on minor salivary gland biopsy; and laboratory evidence of the following: RF+, ANA+, or Ro+ or La+ antibodies.
Which antibodies are frequently present in pediatric Sjogren syndrome?
Ro+ (SSA; 70%) and La+ (SSB; 50%) antibodies
How do most pediatric cases of Sjogren syndrome present?
Most cases present with recurrent parotitis and keratoconjunctivitis sicca.
What is the ratio of males:females diagnosed with Sjogren syndrome?
The female:male ratio is 3:1
Differentiate primary from secondary Sjogren syndrome.
Primary Sjogren syndrome is defined as an isolated disorder, while secondary SS is associated with another autoimmune disease. Some children with a diagnosis of primary SS go on to develop another autoimmune disorder, most commonly SLE.
What is the recommended treatment for symptomatic patients with Sjogren syndrome?
Treat with agents that increase moisture: artificial tears, artificial saliva, and pilocarpine to stimulate exocrine secretions. Chewing sugarless gum can help stimulate salivary flow as well.
How often should patients with Sjogren syndrome be seen by the dentist?
At least 2-3x/year, due to marked increase in caries from decreased salivation.
Which malignancies are patients with Sjogren syndrom at risk for?
Lymphoma, especially mucosal associated lymphoid tissue lymphoma (MALT) and non-Hodgkin B-cell lymphomas.
Infants born to mothers with Sjogren syndrome are at risk for development of what condition?
Children born to mothers with Sjogren syndrome who have Ro and/or La antibodies are at risk for developing neonatal lupus.