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
Other diagnostic factors
- cramping abdominal pain, diarrhea, or gastrointestinal ulceration
Risk factors
- age 20-40 years
- family history of Behcet syndrome
- genetic predisposition
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
Emerging tests
- anti-Saccharomyces cerevisiae antibodies
Treatment algorithm
mucocutaneous ulcers
eye involvement
gastrointestinal involvement
central nervous system involvement
major vascular involvement
Contributors
Authors
Yusuf Yazici, MD
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
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.
References
Key articles
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. Abstract
International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990 May 5;335(8697):1078-80. Abstract
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.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Aphthous stomatitis (recurrent)
- Crohn disease
- Ulcerative colitis
More DifferentialsGuidelines
- 2018 update of the EULAR recommendations for the management of Behçet's syndrome
- Criteria for diagnosis of Behcet's disease
More Guidelines
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