Behçet’s Disease

Question 1

Define Behçet’s disease.

Answer 1

A systemic vasculitis which can cause skin and mucosal lesions, uveitis, major arterial and venous vessel disease, and GI and neurological manifestations.

Question 2

Explain the aetiology/risk factors of Behçet’s disease.

Answer 2

Age 20 to 40 years
Family history of Behçet’s syndrome
Genetic predisposition

Question 3

Summarise the epidemiology of Behçet’s disease.

Answer 3

Patients are most commonly from the Middle East, the Mediterranean region, and eastern Asia, with Japan and South Korea leading the list. Behçet’s syndrome is rare in northern Europe and Africa. The prevalence in Turkey is 1 in 250 of the adult population.

Question 4

Recognise the signs of Behçet’s disease on physical examination. Recognise the presenting symptoms of Behçet’s disease.

Answer 4

Increased predisposition in certain ethnic/geographic groups
Oral ulcers
Genital ulcers
Uveitis
Acne lesions
Erythema nodosum

Question 5

Identify appropriate investigations for Behçet’s disease and interpret the results.

Answer 5

Pathergy testing
RF
ANA
Anti-neutrophil cytoplasmic antibodies
HLA-B51
MRI brain with contrast
Colonoscopy
Upper GI endoscopy
High-resolution chest CT
CT angiography of chest
Pulmonary angiography