Sjogren's syndrome

Question 1

Define

Answer 1

Systemic autoimmune disorder resulting in destruction of the exocrine glands, specifically the lacrimal and salivary glands.

Question 2

Epidemiology

Answer 2

Female to male ratio = 9:1

Diagnosis usually after menarche or menopause

Question 3

Aetiology

Answer 3

Genetic associations (causes unknown)

• HLA-B8
• HLA-DR3

Other autoimmune diseases
-SLE
-Rheumatoid arthritis
-systemic sclerosis 
?retroviral molecular mimicry 
infection of the salivary or molecular glands

Question 4

Pathophysiology

Answer 4

Anti-SS-A and anti-SS-B antibodies (both of which are ANA)

Note; SS-A (anti Rho) and SS-B (anti La) are riboproteins, which are proteins in the nucleus and cytoplasm complexed with RNA.

Question 5

Symptoms

Answer 5

Keratoconjunctivitis sicca = dry eyes (pt often use eye drops)

• ‘sand in eyes’ over 3 months
• itching, burning eyes
• sensitive to light and wind

Dry mouth (xerostomia)(pt often need 'wet' food e.g. butter on food)
- leading to secondary dysphagia 

Dry vagina
Dyspareunia
Dry skin and lips
Digestive problems

Fatigue & sleep disturbance
Reduced mobility

Question 6

Signs

Answer 6

Parotid or salivary gland enlargement 
Dry eyes
Dry mouth (xerostomia) or tongue 
Signs of associated conditions  
Dental caries (loss of teeth due to destruction)

Question 7

Main Investigations

Answer 7

Schirmer’s test (measure of tears by placing filter paper in lower conjunctival sac; if less than 5mm of paper is wet after 5 mins, it’s positive for Sjogren’s)
Serology - anti-60 kD Ro and anti-La however this is not diagnostic
Bloods -

Question 8

Other investigations

Answer 8

Decreased sialometry (reduced unstimulated salivary flow for 15 mins)

Minor salivary gland biopsy (mononuclear cell infiltrates)

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