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Behcet's disease

Clinical diagnosis:

  1. Oral or genital ulcers

  2. Skin rashes/lumps or bumps

  3. Family/ancestors from Turkey, Middle East, central Asia

  4. *BRVO (vasculitic changes) with posterior uveitis*

  5. Anterior uveitis with shifting/sterile hypopyon + white eye

  6. Macular retinitis

  7. Smoky vitreous without condensations (vitritis)

  8. ONH neuropathy (risk becoming atrophic) – life threatening! Refer to medics

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Investigations:

  1. HLA-B51

  2. Inflammatory markers – FBC / CRP / ESR

  3. Syphilis serology and Quantiferon-TB Gold test – rule out the differentials

  4. MRI brain – if neurological involvement

  5. FFA – determine areas of ischaemia for treatment

  6. Refer to physician

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Differential diagnosis: TB ; Syphilis


Management:

  1. Anterior uveitis – treat aggressively with topical steroids and mydrilates.

  2. Laser photocoagulation – areas of ischaemia or macular oedema.

  3. Neurology symptoms – physician involvement urgently

  4. For any neurology associated symptoms or vasculitis or macular retinitis – pulsed IVMP then oral prednisolone (long-term). This is followed by immunosuppression.

  5. Immunosuppression – mycophenolate mofetil (common) ; alternatives – cyclosporin or azathioprine.

  6. Biologics – anti TNF agents (infliximab or adalimumab).

  7. Interferon alpha.

  8. RRD – rare – usually after intravitreal steroid treatment or tractional related cause from prolonged vitritis – treat with scleral buckle or PPV

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