Sjögren's syndrome Flashcards

1
Q

Define Sjogren’s syndrome.

A

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

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2
Q

What are the two types of Sjogren’s?

A

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.

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3
Q

Which antibodies are present in Sjogren’s?

A

ANA

Anti-Ro

Anti-La

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4
Q

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

A

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

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5
Q

What is the aetiology of Sjogren’s?

A

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

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6
Q

What are the risk factors for Sjogren’s?

A
  • Female
  • SLE
  • RhA
  • Systemic sclerosis
  • HLA class II markers - A1, -B8, or -DR3/DQ2
  • Some genetic links
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7
Q

What are the clinical features of Sjogrens syndrome?

A
  • 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
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8
Q

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

A

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

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9
Q

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

A
  • Non-Sjogren’s sicca e.g. drug-induced, age-related or idiopathicSLE/RhA/SS
  • Hypothyroid
  • Fibromyalgia
  • Amyloidosis/sarcoidosis
  • Bulimia
  • Salivary gland tumours
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10
Q

What investigations are used for diagnosis of Sjogren’s?

A

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
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11
Q

What criteria is used to diagnose Sjogren’s?

A

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.
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12
Q

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

A

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.

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13
Q

What is the management of Sjogren’s syndrome?

A

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,
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14
Q

Which drugs should be avoided in Sjogren’s patients?

A

Avoid anticholinergics, antihistamines and diuretics.

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15
Q

What are the complications of Sjogren’s?

A
  • 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
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16
Q

What is the prognosis of Sjogren’s?

A

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