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[Behçet's Syndrome]

Theodoros Xenitidis1, Joerg Christoph Henes1

  • 1Abteilung und Lehrstuhl II, Hämatologie/Onkologie/klinische Immunologie/Rheumatologie, Medizinische Universitätsklinik, Universitätsklinikum Tübingen.

Laryngo- Rhino- Otologie
|June 20, 2020
PubMed

Insights

Behçet

Area of Science:

  • Rheumatology and Immunology
  • Vascular Medicine

Background:

  • Behçet's syndrome (BS) is a complex vasculitis with diverse clinical manifestations.
  • Its pathogenesis bridges polygenic autoinflammatory and autoimmune disease characteristics.
  • Recent EULAR recommendations (2018) guide management strategies.

Purpose of the Study:

  • To provide an overview of Behçet's syndrome classification and pathogenesis.
  • To highlight current therapeutic approaches based on affected organs.
  • To discuss recent advancements in BS treatment.

Main Methods:

  • Review of current literature and clinical guidelines for Behçet's syndrome.
  • Analysis of pathogenetic mechanisms.
  • Evaluation of therapeutic options including biologics and targeted therapies.

Main Results:

  • Behçet's syndrome is a variable vessel vasculitis requiring multidisciplinary care.
  • Adalimumab is approved for posterior ocular involvement since 2016.
  • Alternative therapies include infliximab, interferon alfa-2a, IL-1 antagonists, and apremilast.

Conclusions:

  • Therapeutic decisions in Behçet's syndrome are guided by organ involvement.
  • Targeted therapies like adalimumab offer new treatment avenues.
  • Ongoing research aims to further elucidate pathogenesis and optimize treatment protocols.

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