Sjogren's Syndrome

Question 1

Define:

Answer 1

Inflammation and destruction of the exocrine gland.

Can be secondary (associated with other autoimmune disease) and primary

Question 2

Aetiology/risk factors;

Answer 2

UNKNOWN
Lymphocytic infiltration and fibrosis of exocrine glands

HLA DR3 and B8

Associated with:
Autoimmune hepatitis 
PBC
Mysthenia Gravis 
Scleroderma
Rheumatoid arthritis 
SLE
Polymoyositis

Question 3

Epidemiology:

Answer 3

Female to male ration 9:1

40-50 years = peak

Question 4

Symptoms:

Answer 4

Systemic:
Fever 
Fatigue 
Weight loss 
Depression
Polyarthritis 

Dry eyes - keratoconjunctivitis Sicca
Dry mouth - dysphagia
Dry upper airways - dry cough and recurrent sinusitis
Dry skin or hair
Dry vagina - painful sex
Reflux gastritis, oesophagitis and constipation

Question 5

Signs:

Answer 5

Dry mouth or tongue
Parotid and salivary gland swelling
Signs of associated conditions
May get peripheral neuropathy

Question 6

Investigations:

Answer 6

High ESR
High amylase
Hypergammaglobinaemia

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

Schirmer’s Test – measures conjunctival dryness
o A strip of filter paper is placed under the eyelid
o Positive for Sjogren’s syndrome if < 5 mm of the strip is wet after 5 mins

Fluorescein/Rose Bengal Stains
o May show punctate or filamentary keratitis (use slit lamp)

o Reduced parotid salivary flow rate
o Reduced uptake or clearance on isotope scan
• Biopsy - of salivary or labial glands shows focal lymphocytic aggregation