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    Behcet S Disease

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    Rendy
    Behcet's disease is a chronic multisystem inflammatory disease characterized by recurrent oral and genital ulcers and eye, skin, vascular, gastrointestinal, and central nervous system lesions. It is most prevalent along the Silk Road with the highest incidence in countries like Iran. Genetics and environmental factors contribute to its pathogenesis. Diagnosis is based on recurrent oral ulcers plus two of genital ulcers, eye lesions, skin lesions, or positive pathergy test. Treatment involves corticosteroids, colchicine, azathioprine, and anti-TNF agents depending on organ involvement.

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    Behcet S Disease

    Uploaded by

    Rendy
    60 views34 pages
    Behcet's disease is a chronic multisystem inflammatory disease characterized by recurrent oral and genital ulcers and eye, skin, vascular, gastrointestinal, and central nervous system lesions. It is most prevalent along the Silk Road with the highest incidence in countries like Iran. Genetics and environmental factors contribute to its pathogenesis. Diagnosis is based on recurrent oral ulcers plus two of genital ulcers, eye lesions, skin lesions, or positive pathergy test. Treatment involves corticosteroids, colchicine, azathioprine, and anti-TNF agents depending on organ involvement.

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    behcet disease

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    Behcet s Disease

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    Behcet's disease is a chronic multisystem inflammatory disease characterized by recurrent oral and genital ulcers and eye, skin, vascular, gastrointestinal, and central nervous system lesions. It is most prevalent along the Silk Road with the highest incidence in countries like Iran. Genetics and environmental factors contribute to its pathogenesis. Diagnosis is based on recurrent oral ulcers plus two of genital ulcers, eye lesions, skin lesions, or positive pathergy test. Treatment involves corticosteroids, colchicine, azathioprine, and anti-TNF agents depending on organ involvement.

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    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    60 views34 pages

    Behcet S Disease

    Uploaded by

    Rendy
    Behcet's disease is a chronic multisystem inflammatory disease characterized by recurrent oral and genital ulcers and eye, skin, vascular, gastrointestinal, and central nervous system lesions. It is most prevalent along the Silk Road with the highest incidence in countries like Iran. Genetics and environmental factors contribute to its pathogenesis. Diagnosis is based on recurrent oral ulcers plus two of genital ulcers, eye lesions, skin lesions, or positive pathergy test. Treatment involves corticosteroids, colchicine, azathioprine, and anti-TNF agents depending on organ involvement.

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    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 34

    BEHCETs DISEASE

    Azami Ahad.MD.

    Definition:

    Behcet disease is a chronic disease with


    multisystem involvment characterized
    clinically by oral&
    ophthalmologic,neurologic, or GI
    manifest (or some combination of
    these)

    Behcets 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

    Behcets disease
    (Epidemiology in Iran)

    Incidence: 345 / year


    Prevalence: 1.67/10000
    or 1 in 6000
    Shahram et al, Behcets disease (Hamza ed) 1997
    Shahram et al, Behcets disease (Hamza ed) 1997

    Behcet' Disease
    Pathogenesis
    Genetic:
    o HLAB51
    o MICA gene (major histocompatibility complex
    class I chain-related gene A)
    o 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.

    Behcets Disease
    Pathogenesis

    CLINICAL SYMPTOMS

    MAJOR

    Mucous Membrane

    Oral

    Genital

    Skin
    Eyes

    MINOR

    Joints

    CNS

    GI

    Vessels

    Others

    Clinical features:
    Oral aphthae
    Often denote the onset of behcets
    disease and constitute a requisite
    diagnostic features
    3-10 lesions

    2-12 mm, discrete, painful, shallow, round


    or oval red-rimmed
    1-3 wk

    Behcets Disease
    Oral Aphtous

    Behcets Disease

    Genital ulcer:
    Like to oral aphtae

    vaginal, vulvar, scrotum, penis,


    perianal
    very painful and disabling

    Behcets 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
    Sweets syndrome like
    Pyoderma gangrenosum
    Pathergy test

    Behcets Disease
    Acneiform nodules

    Behcets 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

    Behcets Disease
    Hypopyon

    Behcet Disease

    Vascular Manifestation

    Large vessel(arterial and venous)

    DVT most common complication

    Arterial pulmonary aneurysm

    Coronary involvement,pericarditis

    Behcets Disease
    Vascular aneurysm

    Behcets Disease
    Vascular Aneurysm

    Behcets Disease
    Venous Thrombosis

    Behcet Disease
    CNS Manifestations

    30% of western patients


    Stroke
    Aseptic meningitis
    MS like
    Peripheral neuropathy(rare)

    Behcets 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%

    New Simple Way to Use Classification


    Tree for the Diagnosis of Behcets Disease

    F. Davatchi, S. Chitsaz, A.R. Jamshidi, A. Nadji, C. Chams, H. Chams, F. Shahram,


    M. Akbarian, F. Gharibdoost.

    Point System

    Ocular Lesions 5 Point


    Oral Aphthosis 4
    Genital Aphthosis
    3
    Pathergy Phenomenon
    Skin Manifestations 1

    Behcets Disease

    D.D

    prognosis

    Behcet Disease

    Treatment-Aphtous Lesions

    Topical or interalesional steroid


    Oral corticosteroid
    Colchicine
    Dapsone
    Thalidomide
    MTX
    Cyclosporine-A
    Interferon-A

    Behcet Disease

    Treatment-ocular Manifestation

    Corticosteroid
    Cyclosporine-A
    Azathioprine
    Cyclophosphamide
    Chlorambucil
    Methotrexate
    Surgery

    Behcet Disease

    Treatment- Vascular Manifestation

    Aspirin
    Warfarin?
    Corticosteroid
    Azathioprine
    Cyclosporine-A
    Surgery: arterial aneurysm

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