- Understand the aetiology and risk factors
- Be able to recognise the clinical presentation
- Discuss diagnostics and treatment strategies
Behcet's disease is a multisystem, chronic-relapsing vasculitis but its target is predominantly the venous system. It is rare but commoner in the populations emanating from the Middle East, Far East and the Mediterranean.
There are a constellation of typical findings - recurrent oral aphthous ulcerations along with genital ulcers and classical eye inflammatory changes such as uveitis and retinal vasculitis. There are typical skin lesions (erythema nodosum) or a positive pathergy test result. However, the diagnosis is entirely clinical as there is no definitive test. Brain involvement is seen in about a third with stroke and headache. There may be brainstem involvement and meningoencephalitis. Cerebral venous sinus thrombosis may occur.
- Bloods FBC, U&E, LFTs, CRP, ESR
- Pathergy test involves using a sterile needle to cause a small skin lesion which develops as a sign of skin hyperreactivity within 48 hours.
- CT/MRI may show brain lesions. CTV or MRV for suspected CVST.
Management has been with various combinations of drugs such as colchicine, thalidomide, dapsone, azathioprine, interferon-alpha and etanercept as well as oral anticoagulation. In neurosarcoid high dose steroids and immunosuppressives may be used. In aggressive disease cyclophosphamide and methotrexate and interferon-alpha, or azathioprine may be considered. Biological agents may also be considered.
- <A href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3002614/pdf/10.1177_1756285609347123.pdf">Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942</A>