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بسم الله الرحمن الرحيم
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Azami Ahad.MD. Rheumathologist
BEHCET’s DISEASE Azami Ahad.MD. Rheumathologist
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Definition: Behcet disease is a chronic disease with multisystem involvment characterized clinically by oral& ophthalmologic,neurologic, or GI manifest (or some combination of these)
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Behcet’s Disease Epidemiology
Is seen worldwide Highest prevalence in eastern mediterranean, middle East, East Asia (Silk Road Disease) Mean ages: 25-30 M/F=1/1
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(Epidemiology in Iran)
Behcet’s disease (Epidemiology in Iran) Incidence: 345 / year Prevalence: 1.67/10000 or 1 in 6000 Shahram et al, Behcet’s disease (Hamza ed) 1997 Shahram et al, Behcet’s disease (Hamza ed) 1997
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Behcet' Disease Pathogenesis
Genetic: HLAB51 MICA gene (major histocompatibility complex class I chain-related gene A) Neg-associtation with HLA-DR1 ,HLA-DQW1 Immunologic: increased activated natural killer cell, molecular mimicry ,HSPs Enviromental: herpes simplex(HSV) streptococcal Ag, mycobacterial ,staph aur.
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Behcet’s Disease Pathogenesis
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CLINICAL SYMPTOMS MAJOR MINOR Mucous Membrane Skin Eyes Joints CNS GI
Oral Genital Skin Eyes MINOR Joints CNS GI Vessels Others
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Clinical features: Oral aphthae
Often denote the onset of behcet’s disease and constitute a requisite diagnostic features 3-10 lesions 2-12 mm, discrete, painful, shallow, round or oval red-rimmed 1-3 wk
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Behcet’s Disease Oral Aphtous
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Behcet’s Disease Genital ulcer: Like to oral aphtae
vaginal, vulvar, scrotum, penis, perianal very painful and disabling
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Behcet’s Disease Genital Aphtous
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Behcet Disease Articular Manifestations
40-60% Intermittent Asymmetric or symmetric Oligoarthritis knee, wrist ,ankle, elbow Nonerosive arthropathy Sacroiliitis(%10?)
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Behcet Disease Skin manifestations
Erythema nodosum Pseudofolliculitis Papulopustular Acneiform nodules Sweet’s syndrome like Pyoderma gangrenosum Pathergy test
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Behcet’s Disease Acneiform nodules
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Behcet’s Disease Pathergy Test (pustule Formation)
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Behcet Disease Ocular Manifestation
%83 to %95 of men %67 to %73 of women Anterior uveitis Posterior uveitis Panuveitis Hypopyon Retinal vasculitis Secondary glaucoma and cataract
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Behcet’s Disease Hypopyon
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Behcet Disease Vascular Manifestation
Large vessel(arterial and venous) DVT most common complication Arterial pulmonary aneurysm Coronary involvement,pericarditis
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Behcet’s Disease Vascular aneurysm
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Behcet’s Disease Vascular Aneurysm
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Behcet’s Disease Venous Thrombosis
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Behcet Disease CNS Manifestations
30% of western patients Stroke Aseptic meningitis MS like Peripheral neuropathy(rare)
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Behcet’s Disease Stroke
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Behcet Disease GI and Kidney manifestations
Melena Abdominal pain Ulceration Crohne like disease GN Epididymitis MAGIC syndrome (mouth, genital ulceration, inflamed cartilage)
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Behcet Disease Laboratory Feature
Acute phase reactant (ESR, CRP) RF negative Complement normal HLAB51 positive CSF analysis (oligoclonal band, IgG index) Synovial fluid (inflammatory or non inflammatoy Imaging: CT, MRI (brain)
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Behcet Disease Diagnostic Criteria
1-Recurrent oral ulceration (at least three times in one of 12-month period) Plus two of 2-Recurrent genital ulceration 3-Eye lesions 4-Skin lesions 5-Positive pathergy tes Yields a sensitivity of 91% and a specificity of 96%
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Revised International Criteria for Behcets Disease
Oral aphthosis Skin manifestations (pseudofolliculitis, skin aphthosis) Vascular lesions (phlebitis, large vein thrombosis, aneurysm, arterial thrombosis) Positive pathergy test Genital aphthosis Ocular lesions 1 point 2 points
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D.D prognosis
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Treatment oTreatment of Behçet’s Disease
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