Sjögren's syndrome

Question 1

Define Sjögren’s syndrome

Answer 1

Sjogren syndrome is a CHRONIC systemic auto-immune disorder characterised by the presence of dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia) and arthritis as a consequence of lymphocytic infiltration.

Question 2

Explain the aetilogy/risk factors

Answer 2

• Female
• Ages 20-30 and after menopause
• Other autoimmune conditions:
  
  • SLE
  • Rheumatoid Arthritis
  • Systemic Sclerosis
• EBV

Sjogren syndrome can be split into:

• Primary SS (occurs alone)
• Secondary SS (occurs along with another auto-immune diseasesuch as lupus, rheumatoid arthritis, and systemic sclerosis)

Question 3

Summarise the epidemiology of Sjögren’s syndrome

Answer 3

• More common in females

Question 4

Recognise the presenting symptoms of Sjögren’s syndrome

Answer 4

• Dry eyes (keratoconjunctivitis sicca)
• Dry mouth (xerostomia)
• Symmetrical Arthritis/Arthralgia - this arthritis is (Non-erosive)

Question 5

Recognise the signs of Sjögren’s syndrome on physical examination

Answer 5

• Dry scaly tongue
• Dry eyes (may see corneal ulcers)
• No saliva pool
• Blepharitis (eyelid inflammation)
• Enlarged salivary glands (mainly parotid)
• Dental cavities & poor dentition: SS increases risk of oral fungal/bacterial infections

Question 6

Identify appropriate investigations for Sjögren’s syndrome and interpret the results

Answer 6

• positive Schirmer’s test:
  
  • Quantitatively measures tears. A filter paper is placed in the lower conjunctival sac. The test is positive if < 5 mm of paper is wetted after 5 minutes
• anti-Ro (SSA) and anti-La (SSB)
• ANA positive
  Rheumatoid Factor +ve
• Lip biopsy (contains minor slivary glands): shows focal lymphocytic sialadenitis (inflammation of salivary gland)

Question 7

What conditions is SS associated with?

Answer 7

• Non-Hodgkin Lymphoma
• PBC
• SLE
• RA
• Systemic Sclerosis
• Interstial Pulmonary Fibrosis

Question 8

Management

Answer 8

Symptomatic:
* artificial tears
* artificial saliva