Behcet's Syndrome

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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