Behcet Syndrome

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Learning objectives

  • Understand the aetiology and risk factors
  • Be able to recognise the clinical presentation
  • Discuss diagnostics and treatment strategies

Introduction

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.

Clinical

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.

Investigations

  • 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

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.


References