Behcet's Syndrome
Health Promotions
Health Lifestyle Education
Stay up to date on vaccines
Avoid smoking and excess alcohol consumption
Regular physical activity
Healthy diet rich in anti-inflammatory foods
No specific disease prevention screening guidelines because of the rarity of the disease, but after diagnosis, the patients should have regular follow ups and screenings for complications:
Regular monitoring of cardiovascular health due to increased risk of thromboembolic events
Imaging to assess for vascular complications or involvement of other organs
Blood tests to monitor inflammatory markers
Psychological support: screen for anxiety and depression because of chronic nature of the disease
Dental check-ups: monitor and manage oral ulcers
Opthalmologic exams: prevent vision problems
Treatment
Pharmaceutical treatment
TNFa inhibitor
Otezla
DMARDs-azathioprine/methotraxate
Glucocorticoids
Treat by organ system involved
Symptoms
Abdominal pain, bleeding, and diarrhea
Headache, fever, poor balance, disorientation, stroke
Joint swelling and pain
Painful, red, and open genital lesions
Painful sores or raised, tender nodules on skin, especially on lower legs
Blurry vision, eye redness, pain, sensitivity to light, and possible vision loss
Painful sores/ulcers in the mouth, tongue, and back of throat
Signs
Erythema nodosum-like lesions
Aneurysms and blocked/narrowed vessels
Arthritis
Asymmetric and nondestructive
Eye inflammation
Glaucoma
Secondary cataracts
Uveitis
Acne-like lesions on skin
Genital ulcerations commonly on scrotum and vulva
Oral (aphthous) ulcerations
Diagnostic Criteria:
PLUS any two of the following:
Pathergy test
Papule 2 mm or more in size, developing 24-48 hours after pricking the skin with a 20-25 gauge needle
Skin lesions
Erythema nodosum-like lesions; papulopustular skin lesions or pseudofolliculitis with characteristic acneiform nodules
Eye lesions
Anterior or posterior uveitis, cells in vitreous on slit-lamp examination or retinal vasculitis
Recurrent, painful genital ulcerations
Aphthous ulceration or scarring
Recurrent oral ulcerations
Painful aphthous ulcerations with at least 3 episodes in any 12-month period
Pathophysiology
A form of vasculitis: Inflammation of arteries and veins of all sizes
Immune dysregulation: Abnormal response to certain antigens which leads to an increased production of inflammatory cytokines.
Genetics
Variant of HLA-B51 increases chance of disease 6 fold
Epidemiology
Women > Men
Middle and far eastern descent
Appears around 20-30 years old
Etiology
Unknown, but thought to be a combination of genetic and environmental factors
Hormonal factors
Environmental: Viral/bacterial infection
Genetics: HLA-B allele
