Vasculitis that leads to mucocutaneous, ophthalmological, vascular, gastrointestinal, and central nervous system (CNS) manifestations.
Most commonly seen in young people aged 20 to 30 years.
More active and severe in males.
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.
A systemic vasculitis, first described by Hulusi Behcet, a Turkish dermatologist, Behcet's syndrome is most commonly seen in Turkey, Israel, the Mediterranean basin, and eastern Asia (where Japan and South Korea report the most cases). 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.
History and exam
Key diagnostic factors
- increased predisposition in certain ethnic/geographic groups
- oral ulcers
- genital ulcers
- acne lesions
- erythema nodosum
- limited duration of symptoms
- superficial thrombophlebitis
- 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
Other diagnostic factors
- cramping abdominal pain, diarrhoea, or gastrointestinal ulceration
- age 20 to 40 years
- family history of Behcet's syndrome
- genetic predisposition
Investigations to consider
- pathergy testing
- rheumatoid factor
- anti-nuclear antibodies
- anti-neutrophil cytoplasmic antibodies
- lumbar puncture
- MRI, magnetic resonance angiography brain with contrast
- upper gastrointestinal endoscopy
- high-resolution chest CT
- CT angiography of chest
- pulmonary angiography
- anti-Saccharomyces cerevisiae antibodies
central nervous system involvement
major vascular involvement
- Aphthous stomatitis (recurrent)
- Crohn's disease
- Ulcerative colitis
- 2018 update of the EULAR recommendations for the management of Behcet's syndrome
- Criteria for diagnosis of Behcet's disease
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