Sjogren's

Question 1

What are the different classifications within Sjögren’s syndrome?

Answer 1

Sicca- dry eyes and dry mouth

Primary- no other connective tissue disease effects

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

Question 2

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

Answer 2

Diet

Chemicals

Drugs

Infection

Genetics

Question 3

What is mixed connective tissue disease?

Answer 3

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

Question 4

What are the epidemiological features of Sjogren’s?

Answer 4

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

Question 5

What are the systemic effects of Sjogrens?

Answer 5

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

Associated with general fatigue and chronic pain

Question 6

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

Answer 6

Can result in complete heart block and pace maker being required

Question 7

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

Answer 7

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

Question 8

What is the theorised timeline of Sjogrens?

Answer 8

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

Question 9

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

Answer 9

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

Question 10

How does Sjogren’s present visually?

Answer 10

Symmetrical enlargement of major salivary glands
-> usually painless

Question 11

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

Answer 11

Caries risk

Infection risk

Loss of lubrication of mouth and eyes

Loss of taste

Question 12

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

Answer 12

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

Question 13

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

Answer 13

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

Question 14

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

Answer 14

Dry eyes

Dry mouth

Autoantibody findings

Imaging findings

Histopathology findings

Radionucleotide assessment

-> 4+ required for diagnosis

Question 15

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

Answer 15

Past head and neck radiation

Hep C

AIDS

Pre-existing lymphoma

Sarcoidosis

GVHD- gland damage can occur in bone marrow transplants

Anticholinergic use

Question 16

How does ACR-EULAR differ from AECG?

Answer 16

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

*Same exclusion criteria but adds IgG4 disease

Question 17

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

Answer 17

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)

Question 18

How does sjogrens appear on different types of imaging modalities?

Answer 18

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

On ultrasound- these holes give leopard spot appearance

Question 19

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

Answer 19

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

Question 20

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

Answer 20

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

Question 21

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

Answer 21

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)

Question 22

What objective test is used to assess saliva in AECG?

Answer 22

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

<1.5ml in 15 mins gives objective dry mouth

Question 23

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

Answer 23

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

Question 24

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

Answer 24

Ro52 (70%)

Ro60 (40%)

La48 (50%)

Question 25

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

Answer 25

Extractable Nuclear Antigens (ENA) ANA RF

Question 26

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

Answer 26

Labial gland biopsy

Question 27

What is a lymphocytic focus in histopathology?

Answer 27

Collection of >50 lymphocytes around a duct - >1 of these is indicative of sjogren's

Question 28

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

Answer 28

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

Question 29

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

Answer 29

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

Question 30

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

Answer 30

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

Question 31

How is Sjogren's managed?

Answer 31

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

Question 32

What are the complications of sjogren's?

Answer 32

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

Question 33

Why is surgery to reduce gland bulk sialosis not recommended?

Answer 33

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

Question 34

How does a salivary lymphoma tend to present?

Answer 34

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

Question 35

How do dentists manage Lymphoma risk of Sjogren's?

Answer 35

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