🔑 Key Learning

  • Autoimmune destruction of exocrine glands causing dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).
  • Can be primary or secondary (especially with RA or other CTDs).
  • Associated with anti-Ro/SSA and anti-La/SSB antibodies.
  • Complications include lymphoma and Raynaud’s.
  • Diagnosis supported by Schirmer’s test, antibodies, and salivary gland biopsy.

🧬 Pathophysiology

Sjogren’s is an autoimmune condition characterised by lymphocytic infiltration and destruction of exocrine glands (particularly salivary and lacrimal glands), leading to impaired secretion.

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.

   

🧠 Classification

  • Primary Sjogren’s – isolated disease
  • Secondary Sjogren’s – occurs with another autoimmune disease, e.g. rheumatoid arthritis, SLE, systemic sclerosis

đź‘€ Clinical Features

  • Epidemiology: Commonly middle-aged women
  • Constitutional: Fatigue, generalised joint pain
  • Exocrine gland dysfunction:
    • Dry eyes (keratoconjunctivitis sicca) – gritty/foreign body sensation, photophobia
    • Dry mouth (xerostomia) – difficulty chewing, swallowing, speaking; ↑ oral infections
    • Parotid gland swelling
    • Vaginal dryness
    • Photosensitive rash
Figure 222: Secondary Raynaud's syndrome in a patient with Sjogren's syndrome. Intermedichbo, CC BY-SA 3.0.

     

đź’Ą Complications

  • Lymphoma – x50 increased risk 
  • Secondary Raynaud’s syndrome

🧪 Investigations

🔬 Antibodies

  • Anti-Ro (SSA) – positive in ~70% (esp. primary)
  • Anti-La (SSB) – positive in ~30%
  • ANA – positive in 70%
  • Rheumatoid Factor – positive in 50%

🧫 Other

  • Schirmer’s test: measures tear production – < 5mm in 5 mins is diagnostic
  • Labial salivary gland biopsy: lymphocytic infiltrate confirms diagnosis

đź’Š Management

  • Dry eyes – artificial tears
  • Dry mouth – artificial saliva, pilocarpine (muscarinic agonist)
  • General symptoms – hydroxychloroquine can be used for arthralgia/fatigue
  • Monitor for lymphoma and refer if red flag signs

đź“ť Exam Clues & Clinchers

  • Gritty eyes + dry mouth + middle-aged woman → Sjogren’s
    • Sjogren’s + recurrent parotitis + candidiasis → primary SS
    • Long-standing RA + dry mouth/eyes → secondary SS
  • Suspected sjogren’s â†’ Check anti-Ro and anti-La, Schirmer's test
  • High risk for lymphoma