Sjogren Syndrome

Background

• Sjogren syndrome is a chronic autoimmune disorder characterized by exocrine gland dysfunction and dryness of mucosal surfaces (sicca symptoms) usually affecting the eyes and mouth.
• It may occur as a primary disorder (primary Sjogren syndrome), but about one-third of patients have another underlying autoimmune condition (secondary Sjogren syndrome) such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or hypothyroidism.
• Sjogren syndrome is more common in women.

Evaluation

• Sicca symptoms, usually dry eyes (xerophthalmia) and dry mouth (xerostomia), are present in > 95% of patients.
• There are no universally agreed on diagnostic criteria for Sjogren syndrome, but the diagnosis should be suspected in patients with sicca symptoms and ≥ 1 of the following:
  • positive blood test for antibodies to anti-SS-A or anti-SS-B antigen
  • positive salivary gland biopsy with evidence of chronic inflammatory infiltrate in exocrine glands
  • nonspecific and/or organ-specific symptoms indicative of systemic manifestations
• Consider the following initial tests to evaluate sicca symptoms:
  • Schirmer test to assess tear function
  • corneal staining with colorants (Rose bengal, fluorescein) to assess damage to tear meniscus from desiccation to conjunctival epithelium
  • unstimulated whole salivary flow collection to assess oral dryness

Management

• There is no known cure for Sjogren syndrome so the treatment goals are symptom relief and prevention of complications.
• Treatment for sicca symptoms:
  • For ocular dryness (xerophthalmia):
    • Consider volume replacement and lubrication using artificial tears and ophthalmic gels/ointments as a first-line therapeutic approach (Weak recommendation).
    • Consider the use of ophthalmic immunosuppressive-containing drops, such as, cyclosporine (Weak recommendation), and autologous serum eye drops for refractory or severe ocular dryness.
    • Consider systemic pilocarpine or cevimeline.
    • Consider punctal occlusion (plugs, cauterization, or surgery) only in severe disease that is refractory to medical therapies.
  • For oral dryness (xerostomia):
    • Perform a baseline evaluation of salivary gland function prior to initiating treatment.
    • Consider the following as first-line therapeutic options (Weak recommendation):
      • nonpharmacologic stimulation for mild dysfunction, including gustatory stimulants and/or mechanical stimulants
      • pharmacologic stimulation with muscarinic agonists (such as, pilocarpine and cevimeline) for moderate dysfunction
      • saliva substitution for severe dysfunction
    • Adequate hydration and good oral hygiene with frequent dental exams, antimicrobial mouth rinses, and daily fluoride can help prevent caries in patients with reduced salivary flow.
• Consider vitamin D supplementation in all patients.
• Consider follow-up:
  • annually in patients with stable disease limited to mucosal surface
  • every 6 months in patients with systemic, extraglandular manifestations
  • every 3 months in patients with end-organ damage