I. Definition--An autoimmune condition characterized clinically by "Sicca syndrome" (dry eyes aka keratoconjunctivitis sicca, mouth, vaginal mucosa)

II. Etiology

  1. Most commonly occurs in conjunction with other rheumatologic disorders, e.g. Rheumatoid Arthritis
  2. Can also occur in late-stage lymphoma
  3. In AIDS, "diffuse infiltrative lymphocytosis syndrome" ("DILS") can produce Sjogren's-like syndrome
III. Clinical/Pathophysiological Features
  1. In primary form, may include polyarthritis, fatigue, renal tubular acidosis, and other organ involvement
  2. Neurologic features appear in about 20% of pts (variable but may include myelopathy, motor neuron disease, optic neuropathy, and brain involvement--See case study publixhed in Medicine 83:280, 2004)
  3. Involves mononuclear infiltration of lacrimal & salivary glands
  4. Often associated with positive antinuclear antibodies (SS-A and SS-B)
  5. Schirmer's test can detect decreased tear production

III. Treatment

  1. Arthritides can be treated as in Rheumatoid Arthritis
  2. See section on keratoconjunctivitis sicca for treatment of eye symptoms
  3. Artificial saliva has poor pt acceptance (tastes like someone elses's spit)
  4. Cholinergic agonists--Can cause diaphoresis, nausea, rhinirtis, and diarrhea; can worsen asthma and narrow-angle glaucoma
    1. Pilocarpine PO (Salagen) 5mg QID
    2. Cevimeline (Evoxac) 30mg TID