Sjögren's Syndrome

Question 1

What is Sjögren’s syndrome? SS

Answer 1

A chronic inflammatory autoimmune disorders that may be primary or secondary.

It is characterised by diminished lacrimal and salivary gland secretion. (sicca complex)

It is sometimes referred to as dry mucuous disorder.

There is lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary.

Question 2

Epidemiology of SS.

Answer 2

Onset usually in 4th or 5th decade of life

female 9:1 male

Question 3

Common symptoms and signs of SS.

Answer 3

Sicca symptoms - xeropthalmia (dry eyes) and xerostomia (dry mouth) as well as fatigue.

Madfred

Myalgia
Arthralgia
Dry mouth
Fatigue
Raynaud’s
Enlarged parotids
Dry eyes

Question 4

Other glands that can be affected.

Answer 4

Can lead to vaginal dryness, dyspareunia, dryg cough and dysphagia.

Question 5

What other diseases are SS associated with?

Answer 5

RA and SLE

Coeliac disease, Primary biliary cirrhosis, autoimmune thyroid disease.

Question 6

Malignancy risk in SS.

Answer 6

5-10% life time risk of B cell lymphoma.

Question 7

Pregnancy and SS.

Answer 7

If a patient is pregnant and anti Ro/SS-A positive there is a risk of fetal loss, complete heart block in fetus and neonatal lupus syndrome.

Question 8

Investigations done in SS.

Answer 8

Autoantibodies

Schirmer’s test

Rose-Bengal staining to show keratitis

Salivary gland biopsy.

Question 9

Which autoantibodies might be seen in SS?

Answer 9

Anti-Ro and Anti-La in 90% of patients.

ANA

RF and anti-ds DNA may be seen.

Question 10

What is Schirmer’s test?

Answer 10

A standard strip of filter paper is placed on the inside of the lower eyelid.

Wetting of less than 10mm in 5 minutes indicates defective tear production.

Question 11

What is seen on salivary gland biopsy?

Answer 11

Focal lymphocytic infiltration

Question 12

Treatment of SS.

Answer 12

Mainy treat symptoms.

Give artificial tears for dry eyes. Also avoid dry or smoky atmospheres.

Give artificial saliva, sugar free gum/pastilles for dry mouth.

Skin emollients and vaginal lubricants.

Immunosuppressant may be indicated in severe systemic disease.

NSAIDs and hydroxychloroquine for arthralgia and fatigue.

Question 13

Complications of SS.

Answer 13

Conjunctivitis and corneal ulcers

Dental cavities and candida infections

Vaginal candidiasis and sexual dysfunction

Rarely affect other organs:

Pneumonia and bronciectasis

Non-Hodgkins lymphoma

Peripheral neuropathy

Vasculitis

Renal impairment