Behcet disease

Last updated: December 8, 2022

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Behcet disease is a type of variable vessel vasculitis that most commonly affects young adults (20–40 years of age) from the Mediterranean region to eastern Asia. Patients typically present with recurrent, painful oral and/or genital ulcerations; uveitis and erythema nodosum are also common in patients with Behcet disease. Diagnosis is based on clinical features, but diagnostic studies (e.g., Doppler ultrasound, MRA head) are required to assess for end-organ damage and exclude differential diagnoses (e.g., aphthous stomatitis, reactive arthritis). Management is based on the affected organs and disease severity, but often involves immunosuppressive agents (e.g., glucocorticoids, azathioprine) and colchicine.

An immune-mediated systemic vasculitis that can involve arteries and veins of all sizes, characterized by the deposition of immune complexes; commonly manifests with cyclical flares of oral and genital ulceration and uveitis [1]

  • Most commonly affects individuals from the Mediterranean region to eastern Asia, with the highest prevalence observed in Turkey and Japan [1]
  • Peak incidence: 20–40 years of age
  • >

Epidemiological data refers to the US, unless otherwise specified.

  • Possible autoimmune and infectious triggers (e.g., precipitating HSV or parvovirus infection) [2]
  • Strong HLA-B51 association

PATHERGY: Positive pathergy test, Aphthous oral ulcers, Thrombosis (arterial and venous), Hemoptysis (pulmonary artery aneurysm), Eye lesions (uveitis, retinal vasculitis), Recurrent Genital ulcers, Young at presentation (3rd decade)

General principles [1][6]

  • Diagnosis is primarily clinical.
  • Diagnostic criteria may be used to establish a diagnosis.
  • Diagnostic studies may be required to assess for end-organ damage and to exclude differential diagnoses.

Rule out other conditions before starting potentially unnecessary and harmful immunosuppressive therapy.

Neuro-Behcet syndrome, vascular disease (e.g., pulmonary artery aneurysms), and GI disease are the main causes of mortality in Behcet disease and should be promptly identified and treated. [1][6]

Diagnostic criteria

International Study Group diagnostic criteria for Behcet disease [1][8]
Mandatory criterion
  • Recurrent (i.e., ≥ 3 episodes within a 12-month period) oral aphthous ulcers
Additional criteria
  • Recurrent genital ulceration
  • Ocular manifestations (e.g., uveitis, retinal vasculitis)
  • Cutaneous lesions
  • Positive pathergy test after 24–48 hours [6]
A diagnosis may be established in patients who fulfill the mandatory criterion PLUS ≥ 2 of the additional criteria.

Laboratory studies [1][6]

Imaging studies [6]

Consider imaging studies based on suspected conditions.

Clinical features of Behcet disease may also be present in several other conditions, e.g.: [1][6]

The differential diagnoses listed here are not exhaustive.

Approach [3][11][12]

  • Nonsevere disease
    • Consult a rheumatologist and other specialists as required.
    • Choice of therapy is based on the type of lesions.
  • Severe disease : Consult a specialist (e.g., neurology, ophthalmology, surgery) and start treatment early to prevent permanent damage. [12]

Up to one-third of patients with GI involvement require emergency surgery as a result of GI perforation, major bleeding, or obstruction. [12]

Pharmacotherapy [11]

Patients with severe disease usually require aggressive management with a combination of high-dose glucocorticoids and other immunosuppressive agents.

Supportive care

  1. Davatchi F, Chams-Davatchi C, Shams H, et al. Behcet’s disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. 2016; 13 (1): p.57-65. doi: 10.1080/1744666x.2016.1205486 . | Open in Read by QxMD
  2. Habibagahi M PhD, Habibagahi Z Md, Saidmardani SM Md, Sadeghian F Md. No Definite Association between Human Parvovirus B19 Infection and Behçet Disease.. Iranian journal of medical sciences. 2015; 40 (6): p.493-500.
  3. Jagdish R Nair, Robert J Moots. Behcet's disease. Clin Med (Northfield Il). 2017; 17 (1): p.71-77. doi: 10.7861/clinmedicine.17-1-71 . | Open in Read by QxMD
  4. Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease.. Pathology research international. 2012; 2012 : p.690390. doi: 10.1155/2012/690390 . | Open in Read by QxMD
  5. Antonio Greco, Armando De Virgilio, Massimo Ralli, Andrea Ciofalo, Patrizia Mancini, Giuseppe Attanasio, Marco de Vincentiis, Alessandro Lambiase. Behçet's disease: New insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018; 17 (6): p.567-575. doi: 10.1016/j.autrev.2017.12.006 . | Open in Read by QxMD
  6. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol. 2012; 9 (2): p.79-89. doi: 10.1038/nrrheum.2012.156 . | Open in Read by QxMD
  7. Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014; 121 : p.1703-1723. doi: 10.1016/B978-0-7020-4088-7.00110-3 . | Open in Read by QxMD
  8. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease.. Lancet. 1990; 335 (8697): p.1078-80.
  9. Kaya TI. Genetics of Behçet's Disease. Pathology Research International. 2012; 2012 : p.1-6. doi: 10.1155/2012/912589 . | Open in Read by QxMD
  10. Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D. Thoracic Manifestations of Behçet Disease at CT. RadioGraphics. 2004; 24 (3): p.801-808. doi: 10.1148/rg.243035091 . | Open in Read by QxMD
  11. Yazici Y, Hatemi G, Bodaghi B, et al. Behçet syndrome. Nat Rev Dis Primers. 2021; 7 (1). doi: 10.1038/s41572-021-00301-1 . | Open in Read by QxMD
  12. 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 . doi: 10.1136/annrheumdis-2018-213225 . | Open in Read by QxMD

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