The challenging treatment of Behcet’s disease

Abstract

Introduction: The treatment of Behcet’s disease (BD) is mainly guided by expert recommendations, the most recent of which were published by European League Against Rheumatism (EULAR) experts in 2008.

Areas covered: A literature search in PubMed with no time limits using the keywords Behcet’s treatment and the individual medications available on the market for the treatment of autoimmune or systemic inflammatory diseases was performed. All publications retrieved were considered. Good evidence based on randomised trials was found for the efficacy of colchicine and azathioprine (AZA) for milder manifestations of BD and of AZA, cyclosporin A (CSA) and interferon α for ocular disease. There is a small randomised trial on etanercept for mucocutaneous BD, and a larger one recently published on apremilast also for mucocutaneous BD; both were superior to placebo. Case series also provide evidence for the efficacy of TNF-antagonists mainly for severe ocular BD. For CNS manifestations, cyclophosphamide is recommended although evidence is sparse and the use of CSA is not encouraged because it may promote CNS disease (four retrospective case series). IL-1 antagonists and tocilizumab have proven effective in case reports even for severe and refractory manifestations.

Expert opinion: The most promising agents in the future treatment of BD are biologics such as TNF antagonists, interferon α, IL-1 antagonists and IL-6 antagonists, as well as apremilast. Prospective clinical trials are underway.

Keywords:

• Behcet’s disease
• challenges
• review
• treatment

Declaration of interest

Prof Kötter received unrestricted grants, fees or consultancy honoraria from Abbvie, Actelion, Bristol-Myers Squibb, Chugai, Janssen, Novartis, Pfizer, Roche and Servier. She has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Notes

This box summarises key points contained in the article.