Treating the Different Phenotypes of Behçet's Syndrome

Front Immunol. 2019 Dec 6:10:2830. doi: 10.3389/fimmu.2019.02830. eCollection 2019.


Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.

Keywords: Behçet's syndrome; DMARDs; anti-TNF-α; cluster analysis; phenotypes.

Publication types

  • Review

MeSH terms

  • Behcet Syndrome / diagnosis*
  • Behcet Syndrome / etiology
  • Behcet Syndrome / therapy*
  • Blood Vessels / immunology
  • Blood Vessels / metabolism
  • Blood Vessels / pathology
  • Cartilage, Articular / immunology
  • Cartilage, Articular / metabolism
  • Cartilage, Articular / pathology
  • Combined Modality Therapy
  • Disease Management
  • Humans
  • Mucous Membrane / immunology
  • Mucous Membrane / metabolism
  • Mucous Membrane / pathology
  • Phenotype*