Sjogren's syndrome

Pathology

  1. Autoimmune disorder - 90% occur in women, average age 50 years
  2. Intermittent or constatn swelling of one or all of hte salivary glands
  3. Diagnosis (at least 2/3)
    • Keratoconjunctivitis sicca (dry eyes)
    • xerostomia (dry mouth)
    • associated connective tissue disorders
  4. If no associated connective tissue disorders are present, this is known as primary Sjogren's disease
  5. Pathology:
    • lymphocyte-mediated destruction of the exocrine glands secondary to B-cell hyper reactivity and associated loss of suppressor T-cell activity
    • x40 increased risk of developing lymphoma, usually B-cell non-Hodgkin's type
    • Antibodies: anti-salivary Ab, rheumatoid factor, anti-SSA-Ro and anti-SSB-La
  6. Investigations
    • Schirmer's tests for xerophthalmia (strip of filter paper inserted into each fornix and hyposecretion confirmed by wetting of less than 5mm in 5 minutes - normal is 15 mm)
    • Slit-lamp examination of the cornea
    • Lip biopsy for histological examination of the minor salivary glands
  7. Treatment
    • Use of artificial tears and saliva
    • Systemis steroids
    • Careful follow-up due to increased risk of lymphoma