SJOGREN'S SYNDROME

Question 1

What is Sjogren’s syndrome?

Answer 1

A chronic autoimmune disease in which the body’s immune system destroys the exocrine glands, specifically the salivary and lacrimal glands, that produce saliva and tears, respectively.

Question 2

What is Sjogren’s syndrome?

Answer 2

A chronic autoimmune disease in which the body’s immune system destroys the exocrine glands, specifically the salivary and lacrimal glands, that produce saliva and tears, respectively.

Question 3

What are the main symptoms of primary Sjogren’s syndrome?

Answer 3

Dry eyes (keratoconjunctivitis sicca)
Dry mouth (xerostomia)
Fatigue
Sometimes dry skin or vagina can be a problem.

Question 4

What is genetic association with Sjorgren’s syndrome?

Answer 4

HLA-B8

HLA-DR3

Question 5

What are the main symptoms of primary Sjogren’s syndrome?

Answer 5

Dry eyes (keratoconjunctivitis sicca)
Dry mouth (xerostomia)
Fatigue
Enlargement of the of salivary and parotid glands
Sometimes dry skin or vagina can be a problem

Question 6

What is genetic association with Sjorgren’s syndrome?

Answer 6

HLA-B8

HLA-DR3

Question 7

What systemic symptoms or complications may be experienced in a minority of Sjogren’s syndrome?

Answer 7

Athralgia
Raynaud's phenomenon
Dysphagia
Other organ-specific autoimmune diseases including thyroid disease and myasthenia gravis
Renal tubular

Question 8

What systemic symptoms or complications may be experienced in a minority of Sjogren’s syndrome?

Answer 8

Athralgia
Raynaud’s phenomenon
Dysphagia
Other organ-specific autoimmune diseases including thyroid disease and myasthenia gravis
Renal tubular defects causing diabetes insipidus (very rare)
Pulmonary diffusion defects and fibrosis
Polyneuropathy
Vasculitis
Increased incidence of non-Hodgkin’s B cell lymphoma

Question 9

What investigations might be ordered to further explore someone who presents with the symptoms of Sjogren’s syndrome?

Answer 9

Schirmer tear test
Rose Bengal staining
Autoantibody tests

Question 10

What is the Schirmer tear test with regard to patients with Sjogren’s syndrome?

Answer 10

A standard strip of filter paper is placed on the inside of the lower eyelid. Wetting of less than 10mm in 5 min indicates defective tear production.

Question 11

What is the Rose Bengal staining test with regard to patients with Sjogren’s syndrome?

Answer 11

Rose Bengal staining of the eyes shows punctate or filamentary keratitis in affected patients.

Question 12

In terms of autoantibodies, what might you expect to find in the blood of someone diagnosed with primary Sjogren’s syndrome?

Answer 12

Rheumatoid factor

Anti-nuclear antibodies (ANA): Anti-Ro (SS-A) and Anti-La (SS-B)

Question 13

Which anti-nuclear antibody is more specific to Sjogren’s syndrome? SS-A or SS-B?

Answer 13

SS-B is found

Question 14

Which anti-nuclear antibody is more specific to Sjogren’s syndrome? SS-A or SS-B?

Answer 14

SS-B is found in 35-85% of people with primary Sjogren’s but only 15% of SLE patients.
SS-A is found in 60-90% of people with primary Sjogrens but also in 40-60% of SLE patients.

Question 15

Which anti-nuclear antibody is more specific to Sjogren’s syndrome? SS-A or SS-B?

Answer 15

SS-B is found in 35-85% of people with primary Sjogren’s but only 15% of SLE patients.
SS-A is found in 60-90% of people with primary Sjogrens but also in 40-60% of SLE patients.

Question 16

What is another name for anti-La, an anti-nuclear antibody?

Answer 16

SS-B

Question 17

What is another name for anti-Ro, an anti-nuclear antibody?

Answer 17

SS-A

Question 18

What is another name for anti-Ro, an anti-nuclear antibody?

Answer 18

SS-A

Question 19

What is the main treatment approach to someone with primary Sjogren’s syndrome?

Answer 19

Symptomatic relief with artificial tears and saliva-replacement solutions.
Hydroxychloroquine may help fatigue and arthlagia
Corticosteroids are rarely needed but are used to treat persistent salivary gland swelling or neuropathy.