Sjögren's syndrome Flashcards
Define Sjogren’s syndrome.
Auto-immune disorder characterised by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) due to lymphocytic infiltration of the lacrimal and salivary glands.
What are the two types of Sjogren’s?
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.
Which antibodies are present in Sjogren’s?
ANA
Anti-Ro
Anti-La
How common is Sjogren’s? Who is most affected?
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
What is the aetiology of Sjogren’s?
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
What are the risk factors for Sjogren’s?
- Female
- SLE
- RhA
- Systemic sclerosis
- HLA class II markers - A1, -B8, or -DR3/DQ2
- Some genetic links
What are the clinical features of Sjogrens syndrome?
- 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
What is the risk to the fetus, in a mother with Sjogren’s in pregnancy?
Risk of congenital heart block due to anti-Ro/La attacking the AV node
Differentials for patient presenting with Sjogren’s-like symptoms?
- Non-Sjogren’s sicca e.g. drug-induced, age-related or idiopathicSLE/RhA/SS
- Hypothyroid
- Fibromyalgia
- Amyloidosis/sarcoidosis
- Bulimia
- Salivary gland tumours
What investigations are used for diagnosis of Sjogren’s?
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
What criteria is used to diagnose Sjogren’s?
American-European Consensus Group classification criteria - requires 3 of 4 objective criteria:
- Objective ocular signs: Schirmer’s test, rose bengal testing or lissamine green and fluorescein
- Involvement of salivary gland by functional testing: salivary scintigraphy, parotid sialography
- Anti-Ro ± Anti-La auto-antibodies
- Histopathology on salivary gland biopsy.
What is the lissamine green test and rose bengal staining test, used in Sjogren’s?
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.
What is the management of Sjogren’s syndrome?
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,
Which drugs should be avoided in Sjogren’s patients?
Avoid anticholinergics, antihistamines and diuretics.
What are the complications of Sjogren’s?
- 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