Sjoren's Syndrome

Question 1

Define

Answer 1

Chronic inflammatory autoimmune disorder characterized byinflammation and destruction of exocrine glands (usually salivary and lacrimal glands)

When associated with other autoimmune diseases, Sjögren’s syndrome is termed secondary
Associated with connective tissue disease (RhA, SLE, etc.)

↘ There is lymphocytic infiltration and fibrosis of exocrineglands, especially lacrimal and salivary glands

Question 2

Causes

Answer 2

UNKNOWN

Genetic associations:

HLA-B8

HLA-DR3

Question 3

Associations

Answer 3

Associated autoimmune diseases:

Rheumatoid arthritis

Scleroderma

SLE

Polymyositis

Organ-specific autoimmune diseases (e.g. PBC, autoimmune hepatitis, myasthenia gravis)

Question 4

Epidemiology

Answer 4

onset between 15-65 yearsF:M, 9:1

Question 5

Symptoms

Answer 5

• General – fatigue, fever, ↓weigh, depression
• Keratoconjuntivitis sicca (dry eyes), ↓tear production,

gritty and sore eyes

• Xerostomia (dry mouth), ↓salivation, ±secondarily

dysphagia

• Dry upper airways: dry cough, recurrent sinusitis
• ↓GI mucus secretion – symptoms of reflux oesophagitis

gastritis, constipation

• Rarely: dry skin/hair, dry vagina, dyspareunia (painful

sex)

Question 6

Signs

Answer 6

Parotid or salivary gland enlargement

Dry eyes

Dry mouth or tongue

Signs of associated conditions

Question 7

Investigations

Answer 7

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 glands