Current pharmacological solutions for Behçet’s syndrome

ABSTRACT

Introduction

Behçet’s syndrome (BS) has a heterogeneous clinical phenotype, and its clinical manifestations may respond differently to drugs commonly used to treat BS. The type, dose, and duration of immunomodulatory, immunosuppressive, and biologic agents should be tailored individually.

Areas covered

We reviewed the literature for articles on BS management that were published until June 2022 and summarized the management options in BS for each type of organ involvement. We aimed to cover all currently available pharmacological agents used in BS, as well as surgical and interventional options, focusing on recent evidence.

Expert opinion

The management aims in BS are to preserve function and quality of life and to avoid damage. The choice of treatment modalities depends on the organs that are actively involved, the severity of that involvement, and prognostic factors. A treat-to-attack strategy would help improve long-term outcomes in BS.

KEYWORDS:

• Behçet’s syndrome
• biologics
• immunosuppressives
• management
• small molecules
• treatment

Article highlights

• Behçet’s syndrome is a highly heterogeneous disease with different organ involvement and its treatment should be tailored according to the type of organ involvement and prognostic factors.

• Rapid and sustained suppression of the inflammation should be aimed for, especially when the involvement is in the major organs.

• In addition to conventional immunomodulatory and immunosuppressive drugs, biological therapies, especially tumor necrosis factor-alpha inhibitors and small molecules, have an increasing role in the treatment of Behçet’s syndrome.

This box summarizes key points contained in the article.

Declaration of interest

Y Ozguler has received speaker fees from UCB Pharma, Novartis and Pfizer. G Hatemi has received grants/research support from Celgene, and speaker fees from AbbVie, Celgene, Novartis and UCB Pharma. SN Esatoglu has received speaker fees from UCB Pharma, Roche, Novartis, Pfizer and Merck Sharp & Dohme. The authors have 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 apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Abbreviations

BS: Behçet syndrome; DOA: direct anticoagulants; IL: interleukin; LUs: leg ulcers; RCTs: randomized controlled trials; TNF: tumor necrosis factor-alpha

Additional information

Funding

This paper was not funded.