Sjogrens Syndrome

Question 1

How can Sjögren’s syndrome be classified?

Answer 1

Sicca syndrome - partial sjogrens

Primary Sjogrens
- no connective tissue disease

Secondary sjogrens
- CT disease present
- SLE: RA, scleroderma etc.

Question 2

Aeitiology of sjogrens?

Answer 2

Genetic predisposition
- anti Ro, La antibodies
- associative not causative

Women
- 10:1

Question 3

Consequences of Sjögren’s syndrome?

Answer 3

Gradual loss of salivary / lacrimal gland tissue through inflammatory destruction

Enlargement of major salivary glands- usually painless and symmetrical

Increased risk of
- lymphoma

Oral and ocular effects due to loss of saliva

Question 4

Describe the immunopathology of sjogrens

Answer 4

Large amounts of T-lymphocytes around the ducts (foci)
- damages cells due to interraction between T, B and cytokines

More than one foci = diagnosis

Question 5

What is the summary of the ACR-EULAR guidelines for sjogrens?

Answer 5

Focus score >1 (weight 3)

Anti Ro antibodies present (weight 3)

Objective measure of salivary and lacrimal flow (weight 1)

Ultrasound of salivary glands (weight 1)

Question 6

What is the summary of the AECG guidelines for sjogrens?

Answer 6

Oral
- Daily feeling of dry mouth for >3 months
- Recurrent swelling of salivary glands as an adult
- Frequently drink liquid to aid swallowing of dry foods
- abnormal unstimulated whole salivary flow <1.5ml in 15 mins

Ocular
- persistent troublesome dry eyes >3 months
- recurrent sensation of sand / gravel in eyes
- tear substitutes used >3 times per day

Question 7

What would a typical positive labial gland biopsy for sjogrens show?

Answer 7

Collection of >50 t lymphocytes around a duct = foci
- Focus score > 1

Generalised lymphocytic infiltrate

Question 8

Order of tests for sjogrens?

Answer 8

First look in patients mouth

Least harmful tests first
- salivary flow >/<1.5ml in 15 mins - unstimulated whole saliva
- anti Ro antibody blood test
- ultrasound salivary scan
- mri of major salivary glands (if positive diagnosis to use as baseline lymphoma scan)

Then labial gland biopsy

Question 9

What tests may be the only positive tests in early sjogrens?

Answer 9

Labial gland biopsy

Anti Ro tests

Question 10

How manage an existing sjogrens pt?

Answer 10

Oral health paramount

• 5000ppmF toothpaste, FV, diet advice
• symptomatic tx of oral dryness
• ?salivary stimulants

Question 11

How tx an early presentation sjogrens with no dry mouth yet?

Answer 11

Liaise with rheumatologist
- consider immune modulating tx such as methotrexate