Behçet’s disease-Short note

Behçet’s disease

• Idiopathic multisystem disease 
• More common in men
• Occurs in 3rd - 4th decade
• Highest incidence in Mediterranean region and Japan
• Associated with HLA-B5

Aetiology : Unknown

Various bacteria and viruses suggested

No good evidence to suggest any of them

Perpetuated by autoimmune response and CD4 + T-cells

Tumour necrosis factor (TNF) thought to be important

Oral aphthous ulceration – 100%

Genital ulceration – 90%

Skin lesions – 80%

Erythema Nodosum

• Acneiform
• Uveitis 70% (inflam. of iris, ciliary body or choroid)

 

CNS involvement – strokes, fits

Major vessels eg superior Vena cava obstruction

Increased skin response to trauma eg blood taking

Ocular Features

Acute iritis

• Pain, redness & ¯VA
• Flare (PTN exudation)
• Inflammatory cells in anterior chamber
• KPs (Inflammatory cells at posterior surface of cornea)

 

Recurrent hypopyon

• (Fluid level of WBC)
• The red or white eye

Marked inflammation of the eye

Retinal vasculitis and haemorrhage (inflam. of retinal vessels)

Occlusive periphlebitis (venous sheathing &  occlusion)

Retinal microinfarcts

Very damaging to vision: retinal damage and optic nerve atrophy

Cataract or glaucoma

Treatment

• Systemic Steroids
• Systemic immunosuppressive agents
• Interferon-alpha may have immunodulating effects
• Anti-TNF monoclonal antibodies may be of help