Behcet's syndrome

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 9 Apr 2025

Last updated: 21 Mar 2024

Behcet's syndrome is a vasculitis with mucocutaneous, ophthalmological, vascular, gastrointestinal, and central nervous system (CNS) manifestations.

Most commonly seen in young people aged 20-30 years.

More active and severe in men.

Azathioprine and other immunosuppressive therapies are used when severe manifestations such as eye, vascular, and CNS involvement are present.

In many patients, disease activity decreases with time, and this natural course can affect treatment decisions about the type and duration of medical therapy.

Definition

Behcet's syndrome, a systemic vasculitis first described by Hulusi Behcet - a Turkish dermatologist - is most commonly seen in Turkey, Israel, the Mediterranean basin, and eastern Asia (where Japan and South Korea report the most cases).[1] It can cause skin and mucosal lesions, uveitis, major arterial and venous vessel disease, and gastrointestinal and neurological manifestations. These can present in various combinations and sequences in patients over time.[2]

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
haemoptysis, cough, shortness of breath, or chest pain
eye redness or tearing
impaired speech, balance, or movement

Full details

Other diagnostic factors

cramping abdominal pain, diarrhoea, or gastrointestinal ulceration

Full details

Risk factors

age 20-40 years
family history of Behcet's syndrome
genetic predisposition

Full details

Diagnostic investigations

Investigations to consider

pathergy testing
rheumatoid factor
anti-nuclear antibodies
anti-neutrophil 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

Clinical Associate Professor of Medicine

New York University School of Medicine

Director

Behcet's Syndrome Center

New York University School of Medicine

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

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

Disclosures

PS declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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's disease
- Ulcerative colitis
More Differentials
Guidelines
- 2018 update of the EULAR recommendations for the management of Behçet's syndrome
- Criteria for diagnosis of Behcet's disease
More Guidelines
Log in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer

Behcet's syndrome

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

Investigations to consider

Emerging tests

Treatment algorithm

mucocutaneous ulcers

eye involvement

gastrointestinal involvement

central nervous system involvement

major vascular involvement

Contributors

Authors

Yusuf Yazici, MD

Disclosures

Peer reviewers

Izzet Fresko, MD

Disclosures

Megan E.B. Clowse, MD, MPH

Disclosures

Philip Seo, MD, MHS

Disclosures

References

Key articles

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

Log in to access all of BMJ Best Practice