New and future perspectives in Behçet's syndrome

Bercemhan Sulu1, Gulen Hatemi1,2

1Department of Internal Medicine, Division of Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
2İstanbul University-Cerrahpaşa, Behçet’s Disease Research Center, İstanbul, Türkiye

Keywords: Behçet’s disease, Behçet’s syndrome, TNF inhibitors, treatment, treat-to-target.

Abstract

Behçet's syndrome is a variable vessel vasculitis characterized by a diverse range of clinical manifestations resulting from inflammation involving several organs and systems. While significant progress has been made in understanding the pathogenesis and treatment of Behçet's syndrome, challenges remain in achieving optimal disease control and preventing long-term complications. This review explores recent advances in the management of Behçet's syndrome, with a focus on emerging therapies and future directions. Apremilast, a phosphodiesterase-4 inhibitor, has shown promise in managing mucocutaneous manifestations, particularly oral ulcers. Tocilizumab, an interleukin (IL)-6 receptor inhibitor, has demonstrated efficacy in certain patient populations, especially those with ocular involvement. However, its use in vascular Behçet's syndrome requires careful consideration. Relapses of oral and genital ulcers can be challenging during tocilizumab treatment. Other emerging therapies, such as IL-17 inhibitors, including secukinumab and ixekizumab, IL-12/23 inhibitor ustekinumab, and Janus kinase (JAK) inhibitors, including tofacitinib and baricitinib, are being investigated for their potential to target specific inflammatory pathways. Future research directions include the development of novel therapeutic targets, better use of existing agents by identifying patient populations that would benefit from these, developing better instruments for disease assessment, and a treat-to-target approach in order to improve outcomes and quality of life for patients with Behçet's syndrome.

Citation: Sulu B, Hatemi G. New and future perspectives in Behçet's syndrome. Arch Rheumatol 2024;39(4):511-521. doi: 10.46497/ ArchRheumatol.2024.11049.

Author Contributions

Wrote the manuscript: B.S., G.H.; Revised the manuscript and granted final approval for submission: G.H.

Conflict of Interest

GH received research grants, speaker fees, and/or consulting fees from Abbvie, Amgen, Boehringer Ingelheim, Deva Pharmaceuticals, Novartis, Pfizer, Soligenix, and UCB Pharma.

Financial Disclosure

The authors received no funding for this review.

Data Sharing Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.