Behcet’s

Question 1

What is Behcet’s syndrome?

Answer 1

Complex multisystem disorder with presumed autoimmune mediated inflammation of arteries & veins (but precise aetiology not fully understood)

Question 2

What is classic triad of symptoms in Behcet’s syndrome?

Answer 2

• Oral ulcers
• Genital ulcers
• Anterior uveitis

Question 3

Answer true or false to the following:
- More common in females
- More severe in males
- Affects young adults (20-40yrs)
- Associated HAL B51
- Not related to FH/genetics

Answer 3

• FALSE; more common in men
• True; more common and more severe in men
• TRUE; affects young adults
• TRUE; associated with HLA b51
• FALSE; 30% positive FH

Question 4

Describe features of Behcet’s syndrome

Answer 4

• Classic triad: oral ulcers, genital ulcers, anterior uveitis
• Haem: thrombophlebitis, DVT, Budd Chiari syndrome
• MSK: arthralgia, morning stiffness
• GI: abdo pain, diarrhoea, colitis
• Skin: erythema nodosum, papules & pustules (similar to acne), vasculitit type rash
• Lungs: pulmonary artery aneurysms (rupture can be fatal)
• Neurological involvement: aseptic meningitis, headaches, migraines, memory impairment

Question 5

How is Behcet’s syndrome diagnosed?

Answer 5

No definitive test BUT positive pathergy test is suggestive

Question 6

What is a positive pathergy test?

Answer 6

The pathergy test involves using a sterile needle to create a subcutaneous abrasion on the forearm. This is then reviewed 24 – 48 hours later to look for a weal 5mm or more in size (passmed also says becomes inflamed with small pustule forming). It tests for non-specific hypersensitive in the skin. It is positive in Behçet’s disease, Sweet’s syndrome and pyoderma gangrenosum.

Question 7

Discuss the management of Behcet’s syndrome

Answer 7

MDT management coordinated by rheumatologist:
- Topical steroids for mouth ulcers
- Systemic steroids
- Colchicine for joint pains
- Topical anaesthetics for genital ulcers
- Immunosupressants (e.g. azathioprine)
- Biologics (e.g. infliximab)

Question 8

Discuss the prognosis of Behcet’s syndrome

Answer 8

Behçet’s disease is a relapsing remitting condition. Patients generally have a normal life expectancy and the condition may go in to complete remission. There is an increased mortality with haemoptysis, neurological involvement and other major complications.