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



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