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BEH Ç ET’S DISEASE

BEH Ç ET’S DISEASE. Idiopathic multisystem disease More common in men Occurs in 3 rd - 4 th decade Highest incidence in Mediterranean region and Japan Associated with HLA-B5. BEH Ç ET’S DISEASE Aetiology. Unknown Various bacteria and viruses suggested

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BEH Ç ET’S DISEASE

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  1. 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

  2. BEHÇET’S DISEASEAetiology • 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

  3. BEHÇET’S DISEASE Systemic Involvement (1) Oral aphthous ulceration – 100%

  4. BEHÇET’S DISEASE Systemic Involvement (2) Genital ulceration – 90%

  5. BEHÇET’S DISEASE Systemic Involvement (3) • Skin lesions – 80% • Erythema Nodosum • Acneiform • Uveitis 70% (inflam. of iris, ciliary body or choroid)

  6. BEHÇET’S DISEASE Systemic Involvement (4) • CNS involvement – strokes, fits • Major vessels eg superior Vena cava obstruction • Increased skin response to trauma eg blood taking

  7. BEHÇET’S DISEASEOcular Features (1) • 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

  8. BEHÇET’S DISEASEOcular Features (3) • 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

  9. BEHÇET’S DISEASETreatment • Systemic Steroids • Systemic immunosuppressive agents • Interferon-alpha may have immunodulating effects • Anti-TNF monoclonal antibodies may be of help

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