Pathophysiology

  • Autoimmune mediated destruction of exocrine glands
  • Classification
    • Primary Sjogren’s 
    • Secondary Sjogren’s - rheumatoid arthritis, connective tissue disease
Figure 221: Sjogren's - Autoimmune destruction of exocrine glands manifesting clinically with xerostomia and keratoconjunctivitis sicca (dry eyes). Scientific Animations, CC BY-SA 4.0.

Clinical Features

  • Epidemiology: Typically affects middle aged women
  • Generalised features:
    • Fatigue
    • Diffuse, generalised joint pain
  • Exocrine dysfunction results in: 
    • Keratoconjunctivitis Sicca - dry eyes - a foreign body, gritty sensation and light sensitivity
    • Dry mouth - xerostomia - difficulty chewing, swallowing or talking. 
      • Increased risk of oral candidiasis 
    • Parotitis - facial swelling
    • Vaginal dryness
  • Photosensitive rashes

Complications

  • Lymphoid malignancy ( x 50 risk)
  • Secondary Raynaud's syndrome
Figure 222: Secondary Raynaud's syndrome in a patient with Sjogren's syndrome. Intermedichbo, CC BY-SA 3.0.

Investigations

Antibodies

  • Rheumatoid factor - 50% positive
  • ANA - 70% positive
  • ANTI-Ro (SSA) - 70% Primary SS
  • Anti-La (SSB) - 30%

Other investigations

  • Schirmer’s test - reduced tear film (<5mm after 5 minutes)
  • Labial (lip) salivary gland biopsy - histology -l focal lymphocytic infiltration 
  • Anti-Ro/La positive

Management

  • Dry eyes and mouth - pilocarpine, artificial tears