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BDS4 Oral Medicine > Sjogren's > Flashcards

Sjogren's Flashcards

1
Q

What are the different classifications within Sjögren’s syndrome?

A

Sicca- dry eyes and dry mouth

Primary- no other connective tissue disease effects

Secondary (majority)- Sjogren’s symptoms caused by SLE, RA or scleroderma

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2
Q

What are the potential triggers of autoimmune diseases like Sjogren’s?

A

Diet

Chemicals

Drugs

Infection

Genetics

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3
Q

What is mixed connective tissue disease?

A

Overlap connective tissue disease- any one of these diseases can cause effects of any of the other
- Spectrum: can polarise to one end- RA (joint issues), Scleroderma (elastic tissues), Sjogren’s (affects lacrimal and salivary glands)
- Sjogren’s complications can appear in later stages of SLE/scleroderma

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4
Q

What are the epidemiological features of Sjogren’s?

A

Half of the people who are diagnosed have other connective tissue disease

Mostly females (10:1)

Late diagnosis as early changes are not noticed (inflammatory process in salivary glands is hidden and destruction of tissue is gradual)

Lifespan not affected

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5
Q

What are the systemic effects of Sjogrens?

A

Vasculitic effects in Lungs, kidney, liver, pancreas, blood vessels, nervous system

Associated with general fatigue and chronic pain

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6
Q

What can occur as a result of neonatal lupus if a baby is born to a mother with Sjogren’s?

A

Can result in complete heart block and pace maker being required

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7
Q

What are the factors thought to be involved in aetiology of Sjogren’s?

A

Genetic predisposition- presence of anti Ro/La antibodies (associative not causitive)

Low Oestrogen

Failures of apoptosis- leading to improper exposure of antigens to immune system

Dysregulation of inflammatory process- dendrite cells recruiting abnormal T cells and pro-inflammatory cytokines

EBV- both are associated with lymphoma

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8
Q

What is the theorised timeline of Sjogrens?

A

At birth patient has genetic predisposition, environmental factors influence, then event such as tissue injury triggers disease process, then changes in salivary flow occur and complain about dry mouth to us

-> disease process has been present for many years and gland destruction can be profound and irreversible

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9
Q

What occurs as a result of inflammatory destruction of lacrimal and salivary glands in Sjogren’s?

A

Autoimmune process mediated through T cells which destroys acini within salivary glands causing lack of function

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10
Q

How does Sjogren’s present visually?

A

Symmetrical enlargement of major salivary glands
-> usually painless

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11
Q

What are the effects of lack of saliva and tears in Sjogren’s?

A

Caries risk

Infection risk

Loss of lubrication of mouth and eyes

Loss of taste

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12
Q

What cancers are Sjogren’s patients at increased risk of?

A

Lymphoma
-> esp salivary marginal B cell lymphoma (MALT)

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13
Q

What are the scoring systems for Sjogren’s diagnosis? (no one test gives answer)

A
  • American-European AECG (traditional)
  • ACR-EULAR- easier to apply
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14
Q

What are the diagnostic criteria for sjogren’s as per AECG?

A

Dry eyes

Dry mouth

Autoantibody findings

Imaging findings

Histopathology findings

Radionucleotide assessment

-> 4+ required for diagnosis

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15
Q

What is the exclusion criteria for AECG? (other obvious causes of dry mouth)

A

Past head and neck radiation

Hep C

AIDS

Pre-existing lymphoma

Sarcoidosis

GVHD- gland damage can occur in bone marrow transplants

Anticholinergic use

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16
Q

How does ACR-EULAR differ from AECG?

A

Gives weighting to each finding or symptom (based on evidence)
- Most is given to histopathology score

*Same exclusion criteria but adds IgG4 disease

17
Q

What are the diagnostic criteria for ACR-EULAR and their weighting?

A

Histopathology- focus score>1 (3)

Autoantibodies- anti-RO (3)

Dry eyes and dry mouth as proven by:
Objective salivary flow test (1)
Schirmer test (1)

Ultrasound evidence (1)

18
Q

How does sjogrens appear on different types of imaging modalities?

A

Snowstorm on sialography- loss of acini causes holes to be present which fill up with dye

On ultrasound- these holes give leopard spot appearance

19
Q

What are the subjective oral symptoms in AECG for sjogren’s?

A

Any of these options give positive score:
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

20
Q

What are the subjective ocular symptoms in AECG for Sjogren’s?

A

Any of these options give positive score:
Persistent troublesome dry eyes for >3 months

Recurrent sensation of sand/gravel in the eyes

Tear substitutes used >3 times day

21
Q

What tests are used to check objectively for ocular signs of Sjogren’s in AECG?

A

Schirmer test
- Calibrated filter paper put into lower eyelid fornix- check how wet the paper is after 5 mins (<5mm suggests lacrimal gland issues and Sjogren’s)

Fluorescing tear film assessment by optician is preferred (green stain sticks to cornea- shows where tear film is not present)
- Preferred ocular test (as optician can provide treatment)

22
Q

What objective test is used to assess saliva in AECG?

A

Unstimulated whole salivary flow- patient spits into a tube for 15 mins

<1.5ml in 15 mins gives objective dry mouth

23
Q

Why is presence of anti-ro/la not considered diagnostic for Sjogren’s?

A

These are not diagnostic as they are found in other connective tissue diseases

24
Q

What are the common types of anti-ro/la antibodies found in sjogrens?

A

Ro52 (70%)

Ro60 (40%)

La48 (50%)

25
Q

Which antibodies found in other connective tissue diseases are not associated with sjogren’s?

A

Extractable Nuclear Antigens (ENA)

ANA

RF

26
Q

What type of biopsy is done in Sjorgen’s testing?

A

Labial gland biopsy

27
Q

What is a lymphocytic focus in histopathology?

A

Collection of >50 lymphocytes around a duct

  • > 1 of these is indicative of sjogren’s
28
Q

Why is a labial gland biopsy not done first if it is most predictive of Sjogren’s?

A

Associated with numbness, discomfort and bruising (don’t do until it is likely that this will be the diagnosis)

29
Q

What is the unusual thing about the findings on a labial gland biopsy for Sjogren’s?

A

Signs would be present on the biopsy many years before the patient presents
-> but people are rarely screened for sjogren’s until they start to have symptoms in later life unless rheumatology colleagues suggest this

  • Patients may also have circulating anti-ro at an earlier age
30
Q

What are the steps in testing for sjogren’s in order?

A

Look in patients mouth for signs of dry mouth

Do the least harmful tests first:
UWS in 15 mins - <1.5ml
Anti-Ro antibody
Salivary USS
Baseline MRI of major salivary glands – for comparison for future lymphoma screen

If still equivocal- labial gland biopsy

31
Q

How is Sjogren’s managed?

A

Manage as for dry mouth- diet, OH, F supplementation (5000ppmF toothpaste) to reduce risk of caries and oral infection due to lack of salivary protection

Pilocarpine- licensed salivary stimulant (side effects may outweigh benefit)

If presents early- before glands are totally destroyed, halting immune progress with immune modulating treatment may prevent symptoms in future (difficult call as patient may never go on to have symptoms)
-> Methotrexate and hydroxychloroquine
-> Long term immune modulating treatment may have consequences

32
Q

What are the complications of sjogren’s?

A

Effects of Oral Dryness
-> caries risk
-> issues with denture retention
-> Infection risk
-> Functional issues –speech/swallow

Salivary enlargement - Sialosis
-> can occur at any time – usually permanent

Lymphoma risk- salivary and general

33
Q

Why is surgery to reduce gland bulk sialosis not recommended?

A

Surgical parotidectomy- can risk facial nerve and be difficult to heal (esp in diabetic patients)

34
Q

How does a salivary lymphoma tend to present?

A

Unilateral salivary gland swelling that occurs suddenly
-> can occur 10-20 years after diagnosis

35
Q

How do dentists manage Lymphoma risk of Sjogren’s?

A

Look for changes at routine exams
-> if suspicious refer to OM who can take a new MRI and compare it with baseline

BDS4 Oral Medicine (28 decks)

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