بسم الله الرحمن الرحيم

Azami Ahad.MD. Rheumathologist BEHCET’s DISEASE Azami Ahad.MD. Rheumathologist

Definition: Behcet disease is a chronic disease with multisystem involvment characterized clinically by oral& ophthalmologic,neurologic, or GI manifest (or some combination of these)

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

(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

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.

Behcet’s Disease Pathogenesis

CLINICAL SYMPTOMS MAJOR MINOR Mucous Membrane Skin Eyes Joints CNS GI Oral Genital Skin Eyes MINOR Joints CNS GI Vessels Others

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

Behcet’s Disease Oral Aphtous

Behcet’s Disease Genital ulcer: Like to oral aphtae vaginal, vulvar, scrotum, penis, perianal very painful and disabling

Behcet’s Disease Genital Aphtous

Behcet Disease Articular Manifestations 40-60% Intermittent Asymmetric or symmetric Oligoarthritis knee, wrist ,ankle, elbow Nonerosive arthropathy Sacroiliitis(%10?)

Behcet Disease Skin manifestations Erythema nodosum Pseudofolliculitis Papulopustular Acneiform nodules Sweet’s syndrome like Pyoderma gangrenosum Pathergy test

Behcet’s Disease Acneiform nodules

Behcet’s Disease Pathergy Test (pustule Formation)

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

Behcet’s Disease Hypopyon

Behcet Disease Vascular Manifestation Large vessel(arterial and venous) DVT most common complication Arterial pulmonary aneurysm Coronary involvement,pericarditis

Behcet’s Disease Vascular aneurysm

Behcet’s Disease Vascular Aneurysm

Behcet’s Disease Venous Thrombosis

Behcet Disease CNS Manifestations 30% of western patients Stroke Aseptic meningitis MS like Peripheral neuropathy(rare)

Behcet’s Disease Stroke

Behcet Disease GI and Kidney manifestations Melena Abdominal pain Ulceration Crohne like disease GN Epididymitis MAGIC syndrome (mouth, genital ulceration, inflamed cartilage)

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)

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%

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

D.D prognosis

Treatment oTreatment of Behçet’s Disease