Sjorgsens Syndrome

Question 1

What is Sjorgsens Syndrome

Answer 1

A chronic inflammatory autoimmune condition. Infiltrating and causing fibrosis of exocrine glands, specifically the lacrimal and salivary glands.

It can be primary or secondary (to RA, SLE, Sytemic sclerosis)

Question 2

What will you find on history taking of Sjorgsens Syndrome?

Answer 2

Symptoms:
Reduced tear production - dry eyes, keratoconjunctivits sicca, Dry, itchy, burning, or gritty eyes, red eyes
Reduced salivation - dry mouth, dental caries (cavities)
Parotid swelling, difficulty swallowing/chewing, parotitis
Other glands - vaginal dryness, dry cough, dysphagia, painful sex
Associated symptoms - arthralgia, raynauds, vasculitis (commonly palpale purpura skin rash), lung/liver/kidney problems, fatigue, peripheral neuropathy

Risk factors:
Other auto immune Conditions - thyroid, autoimmune hepatitis, primary biliary cirrhosis)
female gender
SLE/systemic sclerosis
rheumatoid arthritis
HLA type II
age 20s and 30s
post-menopause.

Question 3

What will you find on examination of a patient with Sjorgsens Syndrome?

Answer 3

X

Question 4

What investigations will you order in Sjorgsens Syndrome?

Answer 4

Bedside:
Urinalysis - May show abnormal levels of phosphate, calcium, potassium, glucose as associated with renal tubular acidosis

Bloods:
UnE Looking for electrolyte abnormalities due to renal tubular acidosis.

Special tests:
Schirmers test - filter paper is placed in the lower conjunctival sac. The test is positive if less than 5 mm of paper is wetted after 5 minutes
Anti RO and Anti LA antibodies
Biopsy of affected gland - focal lymphocytic aggregation

Question 5

What is the treatment of Sjorgsens Syndrome

Answer 5

Lifestyle:
Drink lots
Sugar free gum

Medical:
NSAIDS and hydroxychlroroquinine for arthralgia
Immunosuppressants in severe disease
Artificial tears
Salivary substitutes
Treat any vasculitis with corticosteroids and any MSK symptoms with paracetamol