Behçet disease: epidemiology, classification criteria and treatment modalities

Expert Rev Clin Immunol. 2024 Dec;20(12):1437-1448. doi: 10.1080/1744666X.2024.2388693. Epub 2024 Aug 11.

Abstract

Introduction: Behçet disease (BD) is an inflammatory multisystem disorder of unknown etiology, believed to be triggered by infection and environmental factors in genetically predisposed individuals. The significance of understanding BD lies in its impact on global health due to its diverse clinical manifestations and geographical distribution.

Areas covered: This review discusses the epidemiology of BD, emphasizing its prevalence estimated at 10.3 (95% CI, 6.1, 17.7) per 100,000 population, with higher rates observed in regions historically linked to the Silk Route. The criteria for diagnosis are explored, focusing on clinical manifestations that guide healthcare professionals in identifying and managing BD. Additionally, the review encompasses treatment strategies, highlighting TNF-alpha inhibitors as pivotal biologics and newer agents like IL-1 inhibitors and Ustekinumab that broaden the therapeutic options for BD.

Expert opinion: Our work provides insights into the evolving landscape of treatments for BD, emphasizing the expanding role of newer agents alongside established therapies like TNF-alpha inhibitors.

Keywords: Tnf inhibitors; behçet disease; biologics; classification criteria; epidemiology.

Publication types

  • Review

MeSH terms

  • Behcet Syndrome* / diagnosis
  • Behcet Syndrome* / drug therapy
  • Behcet Syndrome* / epidemiology
  • Behcet Syndrome* / therapy
  • Humans
  • Interleukin-1 / antagonists & inhibitors
  • Prevalence
  • Tumor Necrosis Factor-alpha* / antagonists & inhibitors
  • Ustekinumab / therapeutic use

Substances

  • Tumor Necrosis Factor-alpha
  • Interleukin-1
  • Ustekinumab