Sjögren's syndrome

Question 1

Define Sjogren’s syndrome.

Answer 1

Auto-immune disorder characterised by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) due to lymphocytic infiltration of the lacrimal and salivary glands.

Question 2

What are the two types of Sjogren’s?

Answer 2

Sjogren syndrome can occur alone (primary Sjogren syndrome) or along with another auto-immune disease (secondary Sjogren syndrome) such as lupus, rheumatoid arthritis, and systemic sclerosis.

Question 3

Which antibodies are present in Sjogren’s?

Answer 3

ANA

Anti-Ro

Anti-La

Question 4

How common is Sjogren’s? Who is most affected?

Answer 4

9: 1 F>M, usually peaks at 20-30yo but also after menopause
0. 5% of population

Most (60%) have the disease secondary to RhA, SLE or systemic sclerosis

Question 5

What is the aetiology of Sjogren’s?

Answer 5

Unknown

Genetic - HLA class II markers HLA-A1, -B8, or -DR3/DQ2 haplotype in white patients are linked with susceptibility to Sjogren syndrome

Oestrogen +/- androgens may play a role

?EBV link

Question 6

What are the risk factors for Sjogren’s?

Answer 6

• Female
• SLE
• RhA
• Systemic sclerosis
• HLA class II markers - A1, -B8, or -DR3/DQ2
• Some genetic links

Question 7

What are the clinical features of Sjogrens syndrome?

Answer 7

• Fatigue
• Dry eyes- recurrent sensation of gravel, itch or burning >3months, increased light sensitivity; may use tear substitutes more than 3 times a day
• Dry mouth - wake at night to drink, difficulties swallowing or even speaking
• Vasculitic rash - small and medium vessel; most common after exocrine disturbance as above
• Dental caries

Other:

• Oral infections - oral candida x10 more likely
• Polyarthritis - non-erosive
• Parotid swelling
• Lung involvement
• Renal involvement
• PNS neuropathy

Question 8

What is the risk to the fetus, in a mother with Sjogren’s in pregnancy?

Answer 8

Risk of congenital heart block due to anti-Ro/La attacking the AV node

Question 9

Differentials for patient presenting with Sjogren’s-like symptoms?

Answer 9

• Non-Sjogren’s sicca e.g. drug-induced, age-related or idiopathicSLE/RhA/SS
• Hypothyroid
• Fibromyalgia
• Amyloidosis/sarcoidosis
• Bulimia
• Salivary gland tumours

Question 10

What investigations are used for diagnosis of Sjogren’s?

Answer 10

Schirmer’s test - test is positive if less than 5 mm of paper is wetted after 5 minutes

Anti-Ro and La antibodies - their presence is also associated with vasculitis in the disease; found in 90%

Consider:

• Sialometry (shown below) or US of salivary glands
• Salivary gland biopsy
• Urinalysis and U&Es
• Angiography

Question 11

What criteria is used to diagnose Sjogren’s?

Answer 11

American-European Consensus Group classification criteria - requires 3 of 4 objective criteria:

1. Objective ocular signs: Schirmer’s test, rose bengal testing or lissamine green and fluorescein
2. Involvement of salivary gland by functional testing: salivary scintigraphy, parotid sialography
3. Anti-Ro ± Anti-La auto-antibodies
4. Histopathology on salivary gland biopsy.

Question 12

What is the lissamine green test and rose bengal staining test, used in Sjogren’s?

Answer 12

Stain is added and used to visualise ocular surface changes due to insufficient tear flow

NB: Rose bengal staining has been largely supplanted by lissamine green staining, because the former is very painful to patients with keratoconjunctivitis sicca.

Question 13

What is the management of Sjogren’s syndrome?

Answer 13

Conservative:

• Artificial tears and saliva - use liberally, thicker preparation for use overnight
• Drink a lot of water
• Oral pilocarpine/cevimeline - cholinergic which stimulates tear secretion
• Minimise water loss from secretions by evaporation (use of humidifiers; moisturising creams or petroleum jelly on lips to prevent cracking and dryness).
• Special glasses - for severe dryness
• Topical fluoride and avoidance of sugars - prevent tooth decay
• +/- Vaginal lubricants
• Monitor severity using ESSDAI questionnaire

Medical:

• DMARDs - rituximab most promising
• NSAIDs for pain
• IV immunoglobulin - used in neuropathy, severe vasculitis,

Question 14

Which drugs should be avoided in Sjogren’s patients?

Answer 14

Avoid anticholinergics, antihistamines and diuretics.

Question 15

What are the complications of Sjogren’s?

Answer 15

• 50% of patients develop disease in sites other than glands e.g. lungs, liver, kidneys
• Infections of the eyes and mouth more common
• Parotid tumours
• NHL increased risk

Question 16

What is the prognosis of Sjogren’s?

Answer 16

Good overall with life expectancy close to normal unless secondary Sjogren’s