Behcet's Syndrome

Etiology

Unknown, but thought to be a combination of genetic and environmental factors

Genetics: HLA-B allele

Environmental: Viral/bacterial infection

Hormonal factors

Epidemiology

Appears around 20-30 years old

Middle and far eastern descent

Women > Men

Genetics

Variant of HLA-B51 increases chance of disease 6 fold

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.

Diagnostic Criteria:

Recurrent oral ulcerations

Painful aphthous ulcerations with at least 3 episodes in any 12-month period

PLUS any two of the following:

Recurrent, painful genital ulcerations

Aphthous ulceration or scarring

Eye lesions

Anterior or posterior uveitis, cells in vitreous on slit-lamp examination or retinal vasculitis

Skin lesions

Erythema nodosum-like lesions; papulopustular skin lesions or pseudofolliculitis with characteristic acneiform nodules

Papule 2 mm or more in size, developing 24-48 hours after pricking the skin with a 20-25 gauge needle

Signs

Uveitis

Secondary cataracts

Glaucoma

Asymmetric and nondestructive

Symptoms

Painful sores/ulcers in the mouth, tongue, and back of throat

Blurry vision, eye redness, pain, sensitivity to light, and possible vision loss

Painful sores or raised, tender nodules on skin, especially on lower legs

Painful, red, and open genital lesions

Joint swelling and pain

Headache, fever, poor balance, disorientation, stroke

Abdominal pain, bleeding, and diarrhea

Treatment

Treat by organ system involved

Pharmaceutical treatment

Glucocorticoids

DMARDs-azathioprine/methotraxate

Otezla

TNFa inhibitor

Health Promotions

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:

Opthalmologic exams: prevent vision problems

Dental check-ups: monitor and manage oral ulcers

Psychological support: screen for anxiety and depression because of chronic nature of the disease

Blood tests to monitor inflammatory markers

Imaging to assess for vascular complications or involvement of other organs

Regular monitoring of cardiovascular health due to increased risk of thromboembolic events

Health Lifestyle Education

Healthy diet rich in anti-inflammatory foods

Regular physical activity

Avoid smoking and excess alcohol consumption

Stay up to date on vaccines