Behçets Disease

Question 1

Define Behçets Disease

Answer 1

Systemic small vessel vasculitis (vasculitides) that often presents with mucous membrane ulceration and ocular issues

Question 2

Aetiology and risk factors of Behçets Disease

Answer 2

Unknown aetiology
Associated with HLA-B51

RF: 20-40, family history, genetic predisposition

Question 3

Epidemiology of Behçets Disease

Answer 3

Turkey, Greece, Israel and the Mediterranean basin
Mostly seen in the second decade
Males have more severe symptoms and poorer outcomes

Question 4

Symptoms of Behçets Disease

Answer 4

1. Recurrent oral ulcers (moist mucosal surface in the mouth)
2. Genital ulcers (painful and scar, especially on the scrotum)
3. Uveitis

Systemic inflammation: rash, abdominal pain
Acne lesions
Severe inflammation of the eye (hypopyon)
Eye pain, blurry vision, photophobia, photosensitivity
Eye redness or tearing
Memory loss, headache, confusion, fever
Haemoptysis, cough, SOB, chest pain

Question 5

Signs of Behçets Disease

Answer 5

Oral and genital ulcers
Uveitis
Erythema nodosum and other rashes
Pericarditis signs 
CNS involvement: (Stroke, Memory loss, Headache, confusion or fever)
Impaired speech, balance or movement

Question 6

Investigations for Behçets Disease

Answer 6

Pathergy testing: Induration with or without formation of a pustule within 48 hours

Antibodies: exclude RA, necrotising vasculitis
HLA-B51: present

LP: (neuro involvement suspected) hypercellularity; mostly lymphocytosis and less frequently polymorphonuclear cells or pleocytosis

MRI: White matter changes commonly involving midbrain, brainstem, and basal ganglia (large vessel phlebitis)

Colonoscopy: GI symptoms -> inflammation and ulcers
HRCT/CT angiography: haemoptysis -> haemorrhage, aneurysms