Sjogren's Syndrome

Question 1

Definition

Answer 1

Characterised by inflammation and destruction of exocrine glands (usually salivary and
lacrimal glands).

o NOTE: when associated with other autoimmune diseases, Sjogren’s syndrome is termed secondary

Question 2

Aetiology/Risk factors

Answer 2

• UNKNOWN

• Genetic associations:
o HLA-B8
o HLA-DR3

• Associated autoimmune diseases:
o Rheumatoid arthritis
o Scleroderma
o SLE
o Polymyositis
o Organ-specific autoimmune diseases (e.g. PBC, autoimmune hepatitis, myasthenia gravis)

Question 3

Presenting symptoms

Answer 3

• Fatigue
• Fever
• Weight loss
• Depression
• Dry eyes (keratoconjunctivitis sicca) - they will be gritty and sore
• Dry mouth - leads to secondary dysphagia
• Dry upper airways - leads to a dry cough and recurrent sinusitis
• Dry skin or hair
• Dry vagina - may cause dyspareunia
• Reduced GI mucus secretions leads to reflux oesophagitis, gastritis and constipation

Question 4

Signs on physical examination

Answer 4

• Parotid or salivary gland enlargement
• Dry eyes
• Dry mouth or tongue
• Signs of associated conditions

Question 5

Investigations

Answer 5

• Bloods
o High ESR
o High amylase (if salivary glands involved)

• Autoantibodies
o Rheumatoid factor
o ANA
o Anti-ENA (extractable nuclear antigens)

• Schirmer’s Test!!!
o A strip of filter paper is placed under the eyelid
o Positive for Sjogren’s syndrome if < 10 mm of the strip is wet after 5 mins

• Fluorescein/Rose Bengal Stains
o May show punctate or filamentary keratitis

• Other Investigations
o Reduced parotid salivary flow rate
o Reduced uptake or clearance on isotope scan

• Biopsy - of salivary or labial glands