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
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