sjögren’s syndrome

Question 1

What is Sjögren’s syndrome?

Answer 1

A chronic autoimmune disorder where the body’s immune system attacks its own salivary glands and lacrimal glands resulting in dry eyes and dry mouth

Question 2

What is Sicca syndrome?

Answer 2

Dry eyes or mouth but not both

Question 3

What is primary and secondary Sjögren’s?

Answer 3

Primary - no connective tissue disease
Secondary - connective tissue disease present such as SLE, rheumatoid arthritis or scleroderma

Question 4

What is the indidence of SS?

Answer 4

0.2-1.2% of people affected
Female:male ratio is 10:1
Lifespan not affected

Question 5

Describe the systemic involvement of SS

Answer 5

Can affect lungs, kidneys, liver, pancreas, blood vessels and nervous system
Sometimes causes general fatigue and chronic pain
Any features in lupus can also be skin in SS

Question 6

What are the aetiological components of SS?

Answer 6

Genetic and environmental

Question 7

Describe the genetic aetiology of SS

Answer 7

No specific inheritance
Association with Anti-Ro and Anti-La seems genetic
Low oestrogen gives risk of connective tissue disease

Question 8

Describe the environmental aetiology of SS

Answer 8

EBV associated - weak evidence
EBV also associated with lymphoma

Question 9

What are the consequences of SS?

Answer 9

Gradual loss of salivary/lacrimal gland tissue through inflammatory destruction
Enlargement of major salivary glands - usually symmetrical (sialosis)
Increase risk of any lymphoma (5%) and MALT lymphoma
Oral and ocular effects of loss of saliva and tears

Question 10

What are the oral and ocular effects of loss of saliva and tears?

Answer 10

Caries risk
Oral infection risk
Loss of lubricating and taste function associated with saliva
Loss of lubrication of eyes from lack of tear production

Question 11

How is SS diagnosed?

Answer 11

Uses ACR-EULAR (2016) - if pt scores ≥4, they can be diagnosed with SS
Histopathological findings (labial biopsy) - 3
Autoantibody findings (anti-la, anti-ro) - 3
Dry eyes/mouth through objective salivary flow and Schirmer test - 1
Ultrasound - 1
Ocular staining score - 1

Question 12

What are the oral symptoms of SS?

Answer 12

Daily feeling of a dry mouth for >3 months
Recurrent swelling of salivary glands as an adult
Frequently drink liquid to aid swallowing dry foods

Question 13

What are the ocular symptoms of SS?

Answer 13

Persistent troublesome dry eyes for >3 months
Recurrent sensation of sand/gravel in the eyes
Tear substitutes used >3 times a day

Question 14

What are the ocular signs of SS?

Answer 14

Abnormal Schirmer test - <5mm wetting in 5 minutes
Fluorescein tear film assessment - green stain

Question 15

What are the oral signs of SS?

Answer 15

Abnormal unstimulated whole salivary flow (UWS) - <1.5ml in 15 minutes

Question 16

Describe the presence of autoantibodies in SS

Answer 16

Not causative with disease process but associated with the clinical pattern
Anti-Ro and Anti-La antibodies most commonly found associated with Sjögren’s

Question 17

Describe the histopathological findings in SS

Answer 17

Labial gland biopsy:
- collection of >50 lymphocytes around a duct is called lymphocytic focus
- generalised lymphocytic infiltrate is termed non-specific sialadenitis
- the most diagnostic feature of SS as will be positive in early stages, before the pt presents with symptoms
- would be the main test but has complications

Question 18

What is the testing order for SS?

Answer 18

Least harmful tests first - salivary flow rate, antibody testing, ultrasound, baseline MRI of major salivary glands
Labial gland biopsy and anti-ro may be the only positive results in early SS

Question 19

How is SS managed if pt presents with dry mouth and salivary deficit?

Answer 19

Gland function is already low so:
- prevention - diet, OHI, fluoride toothpaste
- symptomatic tx of oral dryness with salivary stimulants

Question 20

How is SS managed if the pt presents early, with no dry mouth yet but active gland disease?

Answer 20

Liaise with rheumatologist
Consider immune modulating tx - hydroxychloroquine and methotrexate

Question 21

How may salivary lymphoma present?

Answer 21

Unilateral gland swelling

Question 22

What is MALT lymphoma?

Answer 22

Mucosa associated lymphoid tissue lymphoma
Caused by salivary marginal B-cells