sjögren’s syndrome Flashcards

1
Q

What is Sjögren’s syndrome?

A

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

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2
Q

What is Sicca syndrome?

A

Dry eyes or mouth but not both

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3
Q

What is primary and secondary Sjögren’s?

A

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

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4
Q

What is the indidence of SS?

A

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

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5
Q

Describe the systemic involvement of SS

A

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

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6
Q

What are the aetiological components of SS?

A

Genetic and environmental

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7
Q

Describe the genetic aetiology of SS

A

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

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8
Q

Describe the environmental aetiology of SS

A

EBV associated - weak evidence
EBV also associated with lymphoma

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9
Q

What are the consequences of SS?

A

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

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10
Q

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

A

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

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11
Q

How is SS diagnosed?

A

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

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12
Q

What are the oral symptoms of SS?

A

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

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13
Q

What are the ocular symptoms of SS?

A

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

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14
Q

What are the ocular signs of SS?

A

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

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15
Q

What are the oral signs of SS?

A

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

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16
Q

Describe the presence of autoantibodies in SS

A

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

17
Q

Describe the histopathological findings in SS

A

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

18
Q

What is the testing order for SS?

A

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

19
Q

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

A

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

20
Q

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

A

Liaise with rheumatologist
Consider immune modulating tx - hydroxychloroquine and methotrexate

21
Q

How may salivary lymphoma present?

A

Unilateral gland swelling

22
Q

What is MALT lymphoma?

A

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