Autoimmune condition that affects the exocrine glands, characterised by lymphocytic infiltrates

Aetiology

• Autoimmune condition
• Higher prevalence in females (9:1), occurs mainly in middle aged women
• Can be a primary condition or can occur secondary to other autoimmune conditions (RA, SLE)

Pathophysiology

• Sjögren syndrome is caused by lymphocyte-mediated autoimmune destruction (type IV hypersensitivity) of minor salivary and lacrimal glands that can lead to atrophy and fibrosis
• Patients typically present with dry eyes and mouth due to the damage to the glands

Clinical features

Symptoms

• Reduced tear secretion (keratoconjunctivitis sicca) - this causes dry, gritty-feeling eyes that may appear red
  • Superimposed bacterial conjunctivitis often occurs
• Dryness of the mouth (xerostomia), which may cause difficulty with swallowing food or talking
• Vaginal dryness, which can cause dyspareunia
• Extraglandular features include systemic upset, arthritis, Raynaud’s and cutaneous vasculitis
• Rarely, other organ involvement in the form of interstitial lung disease, renal disease and neurological disease including seizures can occur

Signs

• Bilateral parotid gland enlargement
• Dental caries, oral candidiasis and fissured tongue often occur

Investigations

• Bloods:
  • Markers of inflammation are usually raised
  • Most patients are ANA positive but this is not specific for Sjögren’s syndrome
  • Rheumatoid factor is positive in 90% of cases
  • Anti-Ro and anti-La autoantibodies are present in 40–90% of patients