sjogrens syndrome

Question 1

what is the general presentation of sjogrens syndrome?

Answer 1

Dry mouth, dry eyes, dry skin, dry vagina, glandular enlargement (esp parotids and submandibular), athralgia, Ryanauds, associated with rheumatoid arthritis

Question 2

define sjogrens syndrome?

Answer 2

chronic inflam and AI disorder characterised by inflammation and destruction of exocrine glands (usually salivary and lacrimal glands) => dry eyes (xerostomia) and dry mouth (keratoconjunctivitis sicca)

Can be primary or secondary associated with other autoimmune diseases

Secondary is associated with CT disorders like SLE, systemic sclerosis etc.

Question 3

what are the genetic associations of sjogren’s syndrome?

Answer 3

HLA-A1

HLA-B8

HLA-DR3

HLA-DQ2

Question 4

which autoimmune diseases are associated with sjogren’s syndrome?

Answer 4

Rheumatoid arthritis

Scleroderma

SLE

Polymyositis

Organ-specific autoimmune diseases (e.g. PBC, autoimmune hepatitis, myasthenia gravis, hashimotos, MS)

Female = RF

Question 5

outline the aetiology of sjogrens syndrome?

Answer 5

UNKNOWN

There is lymphocytic infiltration and fibrosis of exocrine glands - increased risk of nonhodgkins lymphoma

Question 6

outline the epidemiology of sjogren’s syndrome?

Answer 6

Primary is much more common in women– 9:1 ratio

Onset tends to be in 40-50s

Question 7

what are the presenting symptoms of sjogren’s syndrome?

Answer 7

Systemic signs

• Fatigue
• Fever
• Weight loss
• Depression
• Polyarthritis/arthralgia

Dry eyes (keratoconjunctivitis sicca) - they will be grittyand sorewith reduced tear production – redness and sensitive to light and wind

Dry mouth - leads to secondary dysphagia/ difficulty speaking/ altered taste

Dry upper airways - leads to a dry cough and recurrent sinusitis

Dry skin or hair

Dry vagina - may cause dyspareunia

Reduced GI mucus secretions leads to reflux oesophagitis, gastritis and constipation

Question 8

what are the signs of sjogrens syndrome?

Answer 8

Parotid or salivary gland enlargement

Dry eyes

Dry mouth or tongue

Signs of associated conditions

May get peripheral neuropathy

Question 9

what are the investigations for sjogrens syndrome?

Answer 9

Schirmer’s Test – measures conjunctival dryness

• A strip of filter paper is placed under the eyelid
• Positive for Sjogren’s syndrome if < 5 mm of the strip is wet after 5 mins

Autoantibodies

• Rheumatoid factor +ve in 38%
• ANA+ve in 74%
• Anti-Rho +ve in 40% (SSA) and anti-La in 26% (SSB)

Drool test

Sialometry- decreased

Bloods

• High ESR
• High amylase (if salivary glands involved)
• Hypergammaglobulinaemia

Fluorescein/Rose Bengal Stains

May show punctate or filamentary keratitis (use slit lamp)

Question 10

outline the management of sjogrens syndrome?

Answer 10