Sjo-gren's Syndrome

Question 1

What is it?

Answer 1

DEFINITION: characterised by inflammation and autoimmune destruction of exocrine glands (usually salivary and lacrimal (tear) glands).

NOTE: when associated with other autoimmune diseases, Sjogren’s syndrome is termed secondary

characterised by the presence of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) as a consequence of lymphocytic infiltration into the lacrimal and salivary glands

Question 2

Explain the aetiology/risk factors of Sjogren’s syndrome

Answer 2

UNKNOWN

Genetic associations:
HLA-B8
HLA-DR3

Associated autoimmune diseases (occurs with them):
Rheumatoid arthritis
Scleroderma
SLE
Polymyositis
Organ-specific autoimmune diseases (e.g. PBC, autoimmune hepatitis, myasthenia gravis)

More risk factrors;
Female
note that genetic inheritance is weak

Question 3

Recognise the presenting symptoms of Sjogren’s syndrome

Answer 3

Fatigue
Dry eyes
Dry mouth - leads to secondary dysphagia

Some patients;
Dental caries - tooth decay
Increased oral bacterial and fungal infections
Myalgia, Arthralgia
Face pain, Mouth burning
Peripheral neuropathy

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 4

Recognise the presenting signs of Sjogren’s syndrome

Answer 4

Enlarged salivary glands
Dry eyes

Dry mouth or tongue

Signs of associated conditions - i.e.e malar flush if SLE association

Question 5

What are appropriate investigations for Sjogren’s ?

Answer 5

1st line;
1. Schirmer’s Test;
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
2. anti-60 kD Ro and anti-La (SS-A & SS-B) antibodies; positive. Not Ro alone as non specific

Other Investigations;
Sialometry - Reduced salivary flow rate
Reduced uptake or clearance on isotope scan

Biopsy - of salivary or labial glands ; score 1 or above indicative of mononuclear infiltrates

Autoantibodies;
The above and
ANA

Fluorescein/Rose Bengal Stains; scores of 3+

Not sure about the below - laz
Bloods;
High ESR
High amylase (if salivary glands involved)