Behcet's syndrome

Question 1

Pathergy is seen in Behcet’s. What is meant by this?

Answer 1

Pathergy is an exaggerated skin injury occurring after minor trauma such as bump, bruise, needle stick injury

Tested by pricking the skin with a needle and seeing if pustules form.

Question 2

What are the clinical features of Behcet’s disease?

Answer 2

• Oral ulcers
• Genital ulcers - painful and may scar e.g. on scrotum
• Uveitis
• Acne lesions
• Erythema nodosum
• Limited duration of symptoms
• Superficial thrombophlebitis - associated with DVT and pulmonary aneurysm formation
• CNS thrombosis
• Hypopyon - virtually pathognomonic
• Non-erosive arthritis

Question 3

Where geographically is Behcet’s most commonly seen?

Answer 3

Turkey, Israel, the Mediterranean basin, and eastern Asia (where Japan and South Korea report the most cases).

Question 4

How do Behcet’s ulcers differ from herpes?

Answer 4

Unlike herpes ulcers, these lesions are always in the moist mucosal surfaces inside the mouth and do not occur on the outer surfaces of the lip

Question 5

What is hypopyon?

Answer 5

Precipitation of inflammatory cells in the anterior chamber = indicates severe inflammation in the eye

Pathognomonic for Behcet’s

Question 6

What are the risk factors for Behcet’s?

Answer 6

• Age 20-40yrs
• FH of Behcet’s syndrome
• Genetic predisposition - evidence for genetic anticipation (i.e., the next generation gets the symptoms earlier and in a more severe form)

Question 7

What are some complications of Behcet’s?

Answer 7

• Stroke
• Blindness
• Increased risk of neoplasia with long-term immunosuppressant use
• Aneurysms esp pulmonary

Question 8

What investigations are used for Behcet’s diagnosis?

Answer 8

• Pathergy testing - positive in 60%
• HLA-B51
• LP/MRI if neurological changes
• Endoscopy/colonoscopy - exclude Crohn’s
• CT +/- angiography
• anti-Saccharomyces cerevisiae antibodies - elevated when GI symptoms are present
• RF - negative
• ANA - negative
• ANCA - negative

Question 9

What is the management of Behcet’s mouth ulcers?

Answer 9

Oral ulcers –>

• Corticosteroid (i.e., triamcinolone oral paste)
• +/- colchicine
• +/- other immunosuppressants (e.g., azathioprine, hydroxychloroquine, thalidomide)
• +/- apremilast - if resistant
• +/- TNF-a inhibitor (e.g., infliximab, adalimumab, etanercept) if resistant

Question 10

What is the management of eye symptoms in Behcet’s?

Answer 10

Refer to ophthalmology

Azathioprine plus a corticosteroid is a commonly used drug regimen

A TNF-alpha inhibitor (e.g., infliximab or adalimumab) or ciclosporin can also be used

Question 11

What is the prognosis in Behcet’s?

Answer 11

Many patients go into remission with time and treatment.

Unlikely to require life-long treatment

Question 12

What is the MOA of colchicine?

Answer 12

Tubulin disruption, disrupts neutrophil recruitment - anti-inflammatory effects

NOT an immunosuppressant

Question 13

What are the two types of uveitis in Behcet’s?

Answer 13

• Anterior
• Retinal

Question 14

Which HLA is associated with Behcet’s?

Answer 14

HLA-B51