Sjogren's syndrome

Question 1

definition of sjogren’s syndrome

Answer 1

chronic inflammatory autoimmune disorder

Characterized by inflammation and destruction of exocrine glands (usually salivary and lacrimal glands).

infiltration by lymphocytes

When associated with other autoimmune diseases, Sjogren’s syndrome is termed secondary.

Question 2

aetiology of sjogren’s

Answer 2

unknown

linked to HLA-B8, DR3

associated with RA, scleroderma, SLE, polymyositis and organ specific autoimmune diseases eg primary biliary cirrhosis, autoimmune hepatitis, autoimmune thyroid disease, myasthenia gravis

lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands

Question 3

epidemiology of sjogren’s

Answer 3

onset between 15 and 65yrs

female more

Question 4

sx of sjogren’s

Answer 4

fatigue

fever

weight loss

depression

Dry eyes (keratoconjunctivitis sicca): Gritty, sore eyes.

Dry mouth (xerostomia): Dysphagia may result secondarily.

Dry upper airways: Dry cough, recurrent sinusitis.

Dry skin or hair (uncommon).

Dry vagina (uncommon): May cause dyspareunia.

low Gastrointestinal mucus secretion causing symptoms of reflux oesophagitis, gastritis, constipation.

Question 5

signs of sjogren’s

Answer 5

parotid gland or salivary gland enlargement - feel parotid and submandibular, see sublingual

dry eyes

dry mouth or tongue - tongue at top of mouth saliva doesnt pool

signs of associated conditions

systemic signs - polyarthritis/arthraligia, raynauds, lymphadenopathy, vasculitis, lung liver and kidney involvement, peripheral neuropathy, myositis and fatigue

Question 6

Ix for sjogren’s

Answer 6

blood

• high ESR
• high amylase if salivary glands involved

auto-Ab (in preg can cross placenta = fetal heart block)

• Rheumatoid factor
• ANA in 60%
• anti-ENA (anti-La, anti-Ro)
• hypergammaglobinaemia

schirmer’s test

• filter paper strip under eyelid
• +ve if <10mm of strip is wet in 5min

fluorescein/rose bengal stains

• May show punctate or filamentary keratitis (clumps of mucus on the cornea).
• use slit lamp

low parotid salivary rate

low uptake or clearance on isotope scan

biopsy - salivary or labial glands (lower lip) - show focal lymphocytic aggregation

Question 7

associations with Sjogren’s

Answer 7

autoimmune disease - thyroid disease, autoimmune hep, OBC

increased risk of non-Hodgkin’s B cell lymphoma