New therapeutic solutions for Behçet’s syndrome

ABSTRACT

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.

KEYWORDS:

• Behçet’s syndrome
• autoinflammatory diseases
• therapy
• biologics
• uveitis

Article highlights

• Behçet’s syndrome is a systemic inflammatory disorder currently classified at a crossroad between autoimmune and autoinflammatory disorders;

• IL-1 inhibitors currently represent the most studied agents among the latest treatment options for Behçet’s syndrome, proving to be effective, safe and characterized by an overall acceptable retention on treatment;

• Tocilizumab, rituximab, alemtuzumab, ustekinumab, interferon-alpha-2a, and apremilast are all potential promising agents for the future management of refractory Behçet’s syndrome;

• Identifying further treatment options for patients unresponsive to the current drug armamentarium is relevant because of the wide clinical spectrum of features responding to certain specific agents that in turn can worsen other Behçet’s manifestations;

• Further studies are needed to identify tissues and organs responsive to any specific therapy, investigate predictive factors of response, identify the best dosage, and establish differences between monotherapy and combination therapies.

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Declaration of interest

The authors have no 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.