Standard and novel therapeutic approaches to Behçet's disease

Behçet's disease (BD), a systemic inflammatory disorder of unknown aetiology, is characterised by recurrent attacks of oral aphthous ulcers, genital ulcers, skin lesions, uveitis or other manifestations affecting the blood vessels, gastrointestinal tract, and respiratory and central nervous systems. Although the treatment of BD is empirical, in recent years, it has been shown that early and effective treatment of acute inflammatory lesions and prevention of relapses can help to reduce the disease burden and improve outcome. Randomised, controlled trials are limited in BD, but those that have been conducted have documented favourable effects of colchicine, ciclosporin, azathioprine, thalidomide, dapsone, depot methylprednisolone, rebamipide, sucralfate, benzathine benzylpenicillin, interferon-alpha-2a and etanercept for various BD manifestations. Anti-inflammatory and/or immunosuppressive treatments should be tailored according to the disease severity and prognostic factors. More potent drugs, such as azathioprine, ciclosporin, interferon-alpha and infliximab, are effective in the suppression of more severe systemic features as well as mucocutaneous manifestations of BD. Although no randomised, controlled trials are yet available, results of open studies with both interferon-alpha and infliximab are promising for those patients with disease resistant to conventional immunosuppressive treatments. Multicentre, multi-disciplinary and long-term trials aiming to assess the efficacy of interventions in both the treatment of acute inflammatory attacks and the prevention of relapses are required in order to provide more generalisable results that can lead to better management plans.