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Behcet syndrome

Last reviewed: 22 Jul 2025
Last updated: 21 Mar 2024

Summary

Definition

History and exam

Key diagnostic factors

  • increased predisposition in certain ethnic/geographic groups
  • oral ulcers
  • genital ulcers
  • uveitis
  • acne lesions
  • erythema nodosum
  • limited duration of symptoms
  • superficial thrombophlebitis
  • hypopyon
  • stroke
  • eye pain, blurry vision, photophobia, or photosensitivity
  • memory loss
  • headache, confusion, or fever
  • hemoptysis, cough, shortness of breath, or chest pain
  • eye redness or tearing
  • impaired speech, balance, or movement
Full details

Other diagnostic factors

  • cramping abdominal pain, diarrhea, or gastrointestinal ulceration
Full details

Risk factors

  • age 20-40 years
  • family history of Behcet syndrome
  • genetic predisposition
Full details

Diagnostic tests

Tests to consider

  • pathergy testing
  • rheumatoid factor
  • antinuclear antibodies
  • antineutrophil cytoplasmic antibodies
  • HLA-B51
  • lumbar puncture
  • MRI, magnetic resonance angiography brain with contrast
  • colonoscopy
  • upper gastrointestinal endoscopy
  • high-resolution chest CT
  • CT angiography of chest
  • pulmonary angiography
Full details

Emerging tests

  • anti-Saccharomyces cerevisiae antibodies

Treatment algorithm

ACUTE

mucocutaneous ulcers

eye involvement

gastrointestinal involvement

central nervous system involvement

major vascular involvement

Contributors

Authors

Yusuf Yazici, MD
Yusuf Yazici

Clinical Associate Professor of Medicine

New York University School of Medicine

Director

Behcet's Syndrome Center

New York University School of Medicine

NY

Disclosures

YY has received research support from Amgen, Genentech, and Bristol-Myers Squibb. YY is a consultant for Bristol-Myers Squibb Chugai, Hemay, and Amgen. He is an author of some of the references cited in this topic.

Peer reviewers

Izzet Fresko, MD

Associate Professor of Medicine

Department of Rheumatology

Cerrahpasa Medical Faculty

Istanbul

Turkey

Disclosures

IF declares that he has no competing interests.

Megan E.B. Clowse, MD, MPH

Assistant Professor of Medicine

Division of Rheumatology and Immunology

Duke University Medical Center

Durham

NC

Declarações

MEBC has received speaking fees from Abbott Pharmaceuticals.

Philip Seo, MD, MHS

Assistant Professor of Medicine

Division of Rheumatology

Johns Hopkins University School of Medicine

Co-Director

Johns Hopkins Vasculitis Center

Baltimore

MD

Declarações

PS declares that he has no competing interests.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behcet's syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003 Jan;82(1):60-76. Resumo

International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990 May 5;335(8697):1078-80. Resumo

Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018 Jun;77(6):808-18.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
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  • Diretrizes

    • 2018 update of the EULAR recommendations for the management of Behçet's syndrome
    • Criteria for diagnosis of Behcet's disease
    Mais Diretrizes
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