Behcet's disease

• Idiopathic, chronic, multisystem disorder

• Recurrent episodes of acute inflammation

• Commonest ophthalmic manifestation: sight-threatening panuveitis and retinal vasculitis

• Typically affects young adults

• Middle eastern ethnicity

• Family history

• HLA-B51 allele (higher rate of ocular involvement)

  • Common in Japan

• Also the HLA-B12 allele in patients with less severe disease course and mostly mucocutaneous disease

Clinical features

• Anterior uveitis: often with hypopyon
• Posterior uveitis: vitritis, macular oedema, retinal infiltrates, haemorrhage, oedema, occlusive periphlebitis/vasculitis, neovascularisation, capillary leakage
• Systemic: oral aphthous ulcers, genital ulceration, erythema nodosum, pseudofolliculitis, papulopustules, acneiform rash, arthritis, thromboses, bloody diarrhoea, intracranial hypertension, cranial/peripheral neuropathies

Classification criteria

• Must have: recurrent oral ulceration
• And two of
  • Recurrent genital ulceration
  • Ophthalmic lesions
  • Skin lesions
  • Positive pathergy test: sterile pustule appearing 24-48 hours after oblique insertion of a 20G needle

Tests

• Consider MR/MRV brain: Neuro-Behcet’s carries the highest mortality

Management:

• Corticosteroids systemically (including pulsed IVMP for acute flares)
• Azathioprine is often first-line immunomodulatory option
• Also: ciclosporin, MMF, infliximab, methotrexate, tacrolimus