Sjogren's

Question 1

What is Sjogren’s Syndrome

Answer 1

• chronic autoimmune disease
• characterized by lymphocytic infiltration and acinar destruction of lacrimal and salivary glands

Question 2

What is the classification of Sjogren’s

Answer 2

• sicca syndrome
• primary sjogren’s
• secondary sjogren’s

Question 3

What is sicca syndrome

Answer 3

• partial sjogren’s findings
• dry eyes or dry mouth - not both

Question 4

What is primary sjogrens

Answer 4

• no other connective tissue disease

Question 5

What is secondary sjogren’s

Answer 5

• connective tissue disease present
• e.g RA or SLE
• 50% of sjogren patients have another CTD

Question 6

What gender is mostly effected by sjogren’s

Answer 6

• women
• neonatal lupus risk - pregnancy

Question 7

Why does sjogren’s often have a late diagnosis

Answer 7

• present at dry mouth stage
• most acinar tissue lost by now

Question 8

What else can Sjogren’s impact

Answer 8

• vasculitic changes present
• therefore renal, lung and nervous tissue changes are common

Question 9

What is the aetiology of sjogrens

Answer 9

• no supported evidence, all speculative
• genetic
• environment

Question 10

What are geentic associations with sjogrens

Answer 10

• runs in families –> no inheritance patterns however
• low oestrogen - CTD risk
• incomplete cell apoptosis –> antigens improperly exposed
• dysregulation of inflammatory process

Question 11

What virus is associated with sjogrens

Answer 11

EBV

Question 12

What is the consequence of sjogren’s

Answer 12

• gradual loss of salivary and lacrimal tissue
• enlargement of major salivary glands
• malignancy

Question 13

How does the enlargement of the major glands present in sjogrens

Answer 13

• usually symmetrical
• usually painless
• late stage finding

Question 14

What malignancies is a sjogren px at risk of

Answer 14

• increased risk of any lymphoma
• icnreased risk of MALT (b-cell) lymphoma

Question 15

What is the pathogenesis of sjogrens

Answer 15

• lymphocyte infiltration is triggered
• consists of cd4+ cells, b cells, plasma cells
• lymphocytes cluster around ducts, replacing acinar cells
• lymphocytic foci present
• final result is destruction of acini and replacement with dense lymphocytic infiltrate

Question 16

What are the two criterias that can be used for diagnosis

Answer 16

• american european consensus group (2002)
• ACR-EULAR joint criteria (2016)

Question 17

What is the AECG criteria based on

Answer 17

• oral/ocular symptoms
• autoantibodies
• imaging
• radionucleotide assessment
• histopathology

Question 18

How many criteria need to be met for the AECG diagnosis

Answer 18

> 4
1 of the findings should be autoantibody/histopathology

Question 19

What is the ACR-EULAR criteria based on

Answer 19

• labial gland biopsy
• autoantibody test
• abnormal schirmer test
• fluorsecin tear film test
• abnormal unstimualted whole salivary flow
• ultrasound (added in 2020- weighting of 1)

Question 20

What are we looking for in labial gland biopsy

Answer 20

• lymphocytic foci
• > 50 lymphocytes around a duct
• > 1 foci = positive result
• most diagnostic feature

Question 21

What is the weighting of labial gland biopsy

Answer 21

3

Question 22

What is the autoantibody test

Answer 22

• associated antibodies = anti ro and anti la
• not causative
• may also test for ANA and RF - not associated with sjogrens but with other CTD

Question 23

What is the weighting of antibody testing

Answer 23

3

Question 24

What is the schirmer test

Answer 24

• paper is stuck in the eye
• should stimulate tears
• <5mm wetting in 5 mins is abnormal result - indicative of sjogrens

Question 25

What is the fluorescin tear film test

Answer 25

* now preferred * stain will stick to areas where tear film is damaged

Question 26

What is the unstimulated whole salivary flow test

Answer 26

* <1.5ml in 15 mins

Question 27

What is the weighting of ocular and oral testing

Answer 27

1

Question 28

How does sjogren appear on imaging

Answer 28

* sialogram --> snow storm appearance * ultrasound --> leopard print appearaence

Question 29

What is the weighting of ultrasound

Answer 29

1

Question 30

How do we reach a diagnosis via ACR-EULAR

Answer 30

* score of =>4 altogether

Question 31

What order should we do testing

Answer 31

* examination first * least harmful tests first

Question 32

What are the least harmful tests

Answer 32

1. unstimulated whole saliva flow 2. anti-ro antibody 3. salivary ultrasound 4. baseline MRI for future lymphoma screen

Question 33

When should we take a labial gland biopsy

Answer 33

* when the other tests come back negative, likely due to early sjogrens * not done in first instance due to risk of discomfort, numbness and bruising

Question 34

What tests are likely to be +ve in early sjogrens

Answer 34

anti-ro biopsy

Question 35

What is the management of sjogrens

Answer 35

based on if they have a dry mouth or not dry mouth * symptomatic tx + enhanced prevention - damage already one no dry mouth * liaise with rheumatology - CTD * consider immunomodulators e.g hydroxychloroquine, methotrexate

Question 36

Why is use of immunomodulators for sjogrens controversial

Answer 36

* dry mouth symptoms not guarenteed * immunomodulators have their own risk * risk vs benefit

Question 37

What are the complications of sjogrens

Answer 37

* effects of oral dryness * salivary enlargement - sialosis * lymphoma

Question 38

What are the long term effects of oral dryness

Answer 38

* caries * denture retention * infection * speech and swallowing

Question 39

How does sjogren associated salivary enlargement present

Answer 39

* can occur at anytime * usually permanent * reduction surgery possible but not advised

Question 40

Who should sjogren px be screened by for lymphoma

Answer 40

* GDP * increase px awareness of changes that indicate lymphoma * unilateral swelling at any stage is concerning