Sjögren's syndrome

Question 1

Define Sjogren’s syndrome

Answer 1

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

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

Question 2

Explain the aetiology/risk factors of Sjogren’s syndrome

Answer 2

UNKNOWN

Genetic associations:
HLA-B8
HLA-DR3

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

Question 3

Recognise the presenting symptoms of Sjogren’s syndrome

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

Recognise the signs of Sjogren’s syndrome on physical examination

Answer 4

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

Question 5

Identify appropriate investigations for Sjogren’s syndrome

Answer 5

Bloods
High ESR
High amylase (if salivary glands involved)

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

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

Fluorescein/Rose Bengal Stains - May show punctate or filamentary keratitis

Other Investigations
Reduced parotid salivary flow rate
Reduced uptake or clearance on isotope scan
Biopsy - of salivary or labial gland