Behcet syndrome

Last reviewed: 5 Sep 2022
Last updated: 06 Aug 2020

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
More key diagnostic factors

Other diagnostic factors

  • cramping abdominal pain, diarrhea, or gastrointestinal ulceration
Other diagnostic factors

Risk factors

  • age 20-40 years
  • family history of Behcet syndrome
  • genetic predisposition
More risk factors

Diagnostic investigations

Investigations 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
More investigations to consider

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, Celgene, Genentech, and Bristol-Myers Squibb. YY is a consultant for Celgene, Bristol-Myers Squibb, and Amgen.

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

Disclosures

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

Disclosures

PS declares that he has no competing interests.

  • Behcet syndrome images
  • Differentials

    • Aphthous stomatitis (recurrent)
    • Crohn disease
    • Ulcerative colitis
    More Differentials
  • Guidelines

    • 2018 update of the EULAR recommendations for the management of Behcet's syndrome
    • Criteria for diagnosis of Behcet's disease
    More Guidelines
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