Sjorgen's Syndrome

Question 1

What is Sjogren’s syndrome?

Answer 1

Chronic inflammatory autoimmune disorder that affects exocrine glands

Question 2

What characterises Sjogren’s syndrome?

Answer 2

Characterised by immunologically mediated destruction of epithelial exocrine glands, especially the lacrimal (eyes) and salivary glands

Question 3

What is the characteristic feature of Sjogren’s syndrome and where is it seen?

Answer 3

Dryness - eyes, mouth (vagina) - and parotid enlargement.

Question 4

How many types of Sjogren’s syndrome are there?

Answer 4

2 - primary and secondary

Question 5

What is primary Sjogren’s syndrome?

Answer 5

Syndrome of dry eyes (keratoconjunctivitis sicca) in the absence of RA or any of the autoimmune diseases

Question 6

Is primary Sjogren’s syndrome more common in females or males?

Answer 6

Females

Question 7

What is secondary Sjogren’s syndrome?

Answer 7

Associated with connective tissue disease e.g. RA, SLE & Systemic
sclerosis

Question 8

What does sjögren’s syndrome often occur secondary to?

Answer 8

Other auto-immune disorders e.g. SLE, RA, scleroderma, primary biliary cirrhosis.

Question 9

What 2 genes are associated with Sjogren’s syndrome?

Answer 9

HLA-B8/DR3

Question 10

What is the pathophysiology of Sjögren’s syndrome?

Answer 10

1. Immunologically mediated destruction of epithelial exocrine glands
2. VIA lymphocytic infiltration + fibrosis of exocrine glands
3. Especially the lacrimal and salivary glands.

Question 11

Give 3 risk factors for Sjogren’s syndrome.

Answer 11

1. Fx (first degree relative = 7x increased risk)
2. Female sex
3. ≥ 40 YO

Question 12

Give 5 symptoms of Sjögren’s syndrome.

Answer 12

1. Dry eyes
2. Dry mouth.
3. Dryness of skin
4. Dryness of vagina.
5. Fatigue.

Question 13

Why might someone with Sjogren’s syndrome have dry eyes?

Answer 13

Dry eyes (keratoconjunctivitis sicca) - due to decrease in tear production

Question 14

Why might someone with Sjogren’s syndrome have dry mouth?

Answer 14

Dry mouth due to decreased saliva production (xerostomia)

Question 15

In a minority of patients, there may be some systemic symptoms. Give 4.

Answer 15

• Arthralgia and occasional non-progressive polyarthritis - similar to that seen in SLE but less common
• Raynauds phenomenon
• Dysphagia and abnormal oesophageal motility as seen in systemic sclerosis but less common
• Other organ specific autoimmune diseases including thyroid disease, myasthenia gravis, primary biliary cirrhosis, autoimmune hepatitis and pancreatitis
• Renal tubular defects (uncommon) causing nephrogenic diabetes insidious and renal tubular acidosis
• Pulmonary diffusion defects and fibrosis
• Polyneuropathy - fits and depression
• Vasculitis
• Increased incidence of non-Hodgkin’s B-cell lymphoma

Question 16

What investigations might you do in someone who you suspect to have Sjögren’s syndrome? Name 4.

Answer 16

1. Schirmer’s test
2. Rose Bengal staining
3. Laboratory blood tests
4. Salivary gland biopsy

Question 17

Investigations: what is the Schirmer’s test?

Answer 17

Schirmer tear test:
1. Strip of filter paper is placed on the inside of the lower eyelid for 5 mins

2. Wetting of < 10 mm in 5 mins = significant!
   -> Indicates defective tear production -> Sjogren’s diagnosis
3. Healthy adults = tears travel 15mm

Question 18

Investigations: what would you test for in the laboratory blood tests for Sjogren’s syndrome?

Answer 18

Look for serum auto-antibodies e.g. anti-RO, RF, ANA.
Also, raised immunoglobulins and ESR.

Laboratory tests:
1. Raised immunoglobulin + ESR levels
2. Rheumatoid factor is usually positive
3. Antinuclear antibodies (ANA) usually found in 80%
4. Anti-Ro (SSA) antibodies are found in 60-90%

Question 19

Investigations: what is the Rose Bengal staining test?

Answer 19

Rose Bengal staining:
* Staining of the eyes shows punctate or filamentary keratitis
- For lack of salivary secretion i.e. xerostomia

Question 20

What is the treatment for Sjögren’s syndrome?

Answer 20

1. Artificial tears, saliva replacement & vaginal lubricants solutions
2. NSAIDs + HYDROXYCHLOROQUINE
   -> may help with fatigue and arthralgia
3. Corticosteroids are rarely needed
   -> but used to treat persistent salivary gland swelling or neuropathy

Question 21

Give the 3 main complications of Sjogren’s syndrome.

Answer 21

1. Eye infections (conjunctivitis, corneal ulcers)
2. Oral problems (dental cavities, candida infections)
3. Vaginal problems (candidiasis, sexual dysfunction)