Sjorgren's Syndrome

Question 1

Definition

Answer 1

Chronic inflammatory autoimmune condition, may be primary or secondary + associated with connective tissue disease
- Rheumatoid arthritis
- SLE
- Systemic sclerosis

Question 2

Epidemiology

Answer 2

Female
> 40 years

Question 3

Risk factors

Answer 3

Family history
HLAB8/DR3

Question 4

Pathophysiology

Answer 4

Lymphocytic infiltration + fibrosis of exocrine glands especially lacrimal + salivary glands

Question 5

Signs

Answer 5

DRY EYES, DRY MOUTH, DRY VAGINA
Eyes:
- Decreased tear production
- keratoconjunctivitis sicca
Mouth
- Decreased saliva production (xerostomia)
- Parotid swelling
Genitals:
- Vaginal dryness
-Dyspareunia
Throat:
- Dry cough
- Dysphagia
Systemic signs:
- Polyarthritis
- Reynaud’s
- Lymphadenopathy

Question 6

Aetiology

Answer 6

Associated with Anti-Ro and Anti-La autoantibodies

Question 7

Diagnosis

Answer 7

Schirmer’s test = tears travelling <10 mm = SIGNIFICANT (20mm + = normal)
Presence of anti-Ro + anti-La
ANA often +ve

Question 8

Treatment

Answer 8

Artificial tears, saliva and lubricant
Hydroxychloroquine = arthralgia
Immunosuppressants = CICLOSPORIN = severe disease

Question 9

Complications

Answer 9

Non-Hodgkin’s B-cell lymphoma