Sjögren’s Syndrome

Question 1

sicca syndrome

Answer 1

partial Sjögren’s findings

Question 2

Sjögren’s Syndrome
types

Answer 2

primary - no connective tissue disease
secondary - connective tissue disease (SLE, Rheumatoid arthritis, scleroderma)

Question 3

Sjögren’s Syndrome is what type of disease

Answer 3

auto immune

complex
many possible triggers

Question 4

Background Sjögren’s Syndrome

Answer 4

0.2-1.2% (0.5-3 million in the USA) people have this
* Half ALSO have another connective tissue disease

Mostly women – 10:1
* Diagnostic delay due to late presentations
* Lifespan not affected
* Risk of neonatal lupus in baby if pregnancy

Systemic involvement
* Lungs, kidney, liver, pancreas, blood vessels, nervous system
* Sometimes general fatigue and chronic pain

Question 5

aetiology of sjorgren’s syndrome

Answer 5

Speculative Genetic
* Genetic predisposition – runs in families, but no specific inheritance
* Association with anti-Ro and anti-La seems genetic (if they have other sjogren’s symptoms then go onto investigate more)
* low oestrogen risk gives a of getting CT disease – androgens protective?
* Incomplete cell apoptosis leads to antigens being improperly exposed
* Dysregulation of inflammatory process with dendritic AP cells recruiting Band T cell responses and pro-inflammatory cytokines

Speculative Environment
* EBV association – weak evidence – may be reactive rather than causative

Question 6

timeline of sjogrens syndrome

Answer 6

Disease process taken place for years before presenting to clinician with dry mouth and loss of salivary gland process

Currently no screening test that can be carried out for pt which may be likely to develop sjogrens

Question 7

oral consequences of sjogren’s syndrome

4

Answer 7

Gradual loss of salivary/lacrimal gland tissue through inflammatory destruction
* Largely mediated by T lymphocytes

Enlargement of major salivary glands – usually symmetrical
* Usually painless
* Late finding

Increased risk
* Any lymphoma (5% quoted)
* Salivary marginal B-cell (MALT) Lymphoma

Oral and Ocular effects of loss of saliva and tears – most immediate pt effect
* Need intervention for these symptoms (caries)

Question 8

immunopathology of sjogren syndrome

Answer 8

B dots – T lymphocytes – around the ducts and acinar, attracted to area and causing gland destruction. Called lymphocytic foci - more than 50 lymphocytes present in one place, need more than one foci to dx sjorgrens

Interaction between T, B and cytokines causing cell tissue destruction

Question 9

dx of sjogrens

Answer 9

Complex – no single test yet gives ‘the answer’ can be hard for pt to accept

Balance of probabilities from multiple criteria

Different scoring systems in use:
* American-European Consensus Group (2002)
* ACR-EULAR joint criteria (2016

Question 10

AECG 2006dx for sjogren’s

American-European Consensus Group

Answer 10

both subjective and objective symptoms, complex process – many tests and pt opinions

Need 4/6 to have sjogrens dx – must have either histopathology or serology +ve

Also have exclusion criteria to run through
* Dry eyes/mouth - Subjective or objective
* Autoantibody findings (anti-Ro or anti-La)
* Imaging findings (ultrasound (no ionising radiation) or sialogram)
* Radio nucleotide assessment (technetium scan)
* Histopathology findings
* FOUR or more positive criteria for diagnosis

Question 11

ACR-EUCLAR joint criteria 2016 for dx sjogrens

Answer 11

Histopathology findings (Weight 3)
* focus score >1

Autoantibody findings (Weight 3)
* anti-Ro

Dry eyes/mouth (Weight 1)
* objective salivary flow
* Schirmer test

Ultrasound now accepted as well (2020)
Same exclusion criteria as AECG but also IgG4 disease

Question 12

sjogren on sialogram and Ultrasonography

Answer 12

‘snowstorm’

loss of acini holes appearing visible sialogram and ‘leopard spot’ appearance on US

US best as no ionising radiation

Question 13

oral symptoms of sjogrens

3

AECG

Answer 13

• 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 14

ocular symptoms of sjogrens
3

AECG

Answer 14

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

Question 15

eye signs for sjogrens

2

Answer 15

Abnormal Schirmer test
* <5mm wetting in 5 minutes
* Calibrated filter paper - collects tear and wets paper

Fluorescein Tear film assessment
* By optician, part of normal eye test

Question 15

eye signs for sjogrens

2

Answer 15

Abnormal Schirmer test
* <5mm wetting in 5 minutes
* Calibrated filter paper - collects tear and wets paper

Fluorescein Tear film assessment
* By optician, part of normal eye test

Question 16

oral sign for sjogrens

Answer 16

Abnormal UNSTIMULATED whole salivary flow (UWS)
* <1.5ml in 15 mins
* Easy to do chairside

Question 17

what do positive autoantibodies for sjogren’s mean

Answer 17

NOT CAUSATIVE in the disease process
ASSOCIATED with the clinical pattern

Antibodies possible without disease – need clinical and lab findings

Question 18

autoantibodies for sjogrens

Answer 18

anti-Ro and anti-La antibodies

Collection of proteins found in the cell
Different ones found in different patients Ro52 (70%), Ro60 (40%) and La48 (50%)

Other Extractable Nuclear Antigens (ENA) not associated
* ANA and RF not associated with Sjögrens

Question 19

what is the best dx test for sjogrens

Answer 19

labial gland biopsy

but it is invasive

Question 20

postive labial gland bipsy for sjogrens

Answer 20

• Collection of >50 lymphocytes around a duct = Lymphocytic Focus
• Generalised lymphocytic infiltrate is ‘non-specific sialadenitis’
• > 1 Focus Score (FS) consistent with Sjogren’s Syndrome

Thought to be the MOST diagnostic feature on ACR-EULAR criteria

Question 21

tests for sjogren’s syndrome
order

Answer 21

First, look in the patient’s mouth
* Sjogren’s patients complaining of dryness will have a dry mouth
* Early Sjogren’s patients will NOT have a dry mouth, nor complain of one (can be 20-30 years till get this complaint)

Do the least harmful tests first
* UWS (unstimulated whole saliva) in 15 mins - <1.5ml
* Anti-Ro antibody
* Salivary ultra sound scan
* Baseline MRI of major salivary glands – for comparison for future lymphoma screen

If still equivocal do labial gland biopsy
* Risk of area of skin numbness following procedure
* Informed consent needed

LGB and Anti-Ro may be the ONLY positive results in early Sjogren’s – may be asked due to other systemic features from rheumatology to get early dx and thus early tx

Question 22

management of sjogrens syndrome when pt presents with a dry mouth and a salivary deficit

3

Answer 22

Gland function is already very low
* Oral Health needs paramount – diet, OHI, 5000ppm toothpaste
* Symptomatic treatment of oral dryness
* Salivary stimulants - pilocarpine?

Question 23

management of sjogrens syndrom when pt presents early (no dry mouth yet)

Answer 23

no dry mouth yet - but still has active gland disease

Liaise with rheumatologist – multisystem disease

Consider Immune modulating treatment – hydroxychloroquine, methotrexate

Question 24

3 complications of sjogrens affecting dental care

Answer 24

Effects of Oral Dryness
* caries risk, denture retention, infections, functional issues –speech/swallow

Salivary enlargement - Sialosis
* can occur at any time – usually permanent
* Reduction surgery possible but not advised – other health issues, risk to facial nerve

Lymphoma risk
* Salivary lymphoma may present with unilateral gland swelling at any stage
* Increased general lymphoma risk too
* Screening? 10/20/30years after initial dx, long time to be at oral med so generally the GDP with patient awareness