INTRODUCTION: Behçet's syndrome (BS) is a systemic inflammatory disorder 
characterized by a wide range of potential clinical manifestations with no 
gold-standard therapy. However, the recent classification of BS at a crossroads 
between autoimmune and autoinflammatory syndromes has paved the way to new 
further therapeutic opportunities in addition to anti-tumor necrosis factor 
agents.
AREAS COVERED: This review provides a digest of all current experience and 
evidence about pharmacological agents recently described as having a role in the 
treatment of BS, including interleukin (IL)-1 inhibitors, tocilizumab, 
rituximab, alemtuzumab, ustekinumab, interferon-alpha-2a, and apremilast.
EXPERT OPINION: IL-1 inhibitors currently represent the most studied agents 
among the latest treatment options for BS, proving to be effective, safe and 
with an acceptable retention on treatment. However, since BS is a peculiar 
disorder with clinical features responding to certain treatments that in turn 
can worsen other manifestations, identifying new treatment options for patients 
unresponsive to the current drug armamentarium is of great relevance. A number 
of agents have been studied in the last decade showing changing fortunes in some 
cases and promising results in others. The latter will potentially provide their 
contribution for better clinical management of BS, improving patients' quality 
of life and long-term outcome.