BEHCET’s 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)

Behcet’s DiseaseEpidemiology

Is seen worldwide Highest prevalence in eastern

mediterranean, middle East, East Asia (Silk Road Disease)

Mean ages: 25-30 M/F=1/1

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) 1997Shahram et al, Behcet’s disease (Hamza ed) 1997

Behcet' DiseasePathogenesis

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.

Behcet’s DiseasePathogenesis

CLINICAL SYMPTOMS

MAJOR

Mucous Membrane

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 DiseaseOral Aphtous

Behcet’s Disease

Genital ulcer:Like to oral aphtae vaginal, vulvar, scrotum, penis,

perianal very painful and disabling

Behcet’s DiseaseGenital Aphtous

Behcet DiseaseArticular Manifestations

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

Behcet DiseaseSkin manifestations

Erythema nodosum Pseudofolliculitis Papulopustular Acneiform nodules Sweet’s syndrome like Pyoderma gangrenosum Pathergy test

Behcet’s DiseaseAcneiform nodules

Behcet’s DiseasePathergy 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 DiseaseHypopyon

Behcet DiseaseVascular Manifestation

Large vessel(arterial and venous) DVT most common complication

Arterial pulmonary aneurysm

Coronary involvement,pericarditis

Behcet’s DiseaseVascular aneurysm

Behcet’s DiseaseVascular Aneurysm

Behcet’s DiseaseVenous Thrombosis

Behcet DiseaseCNS Manifestations

30% of western patients Stroke Aseptic meningitis MS like Peripheral neuropathy(rare)

Behcet’s DiseaseStroke

Behcet Disease GI and Kidney manifestations

Melena Abdominal pain Ulceration Crohne like disease GN Epididymitis MAGIC syndrome (mouth, genital ulceration, inflamed cartilage)

Behcet DiseaseLaboratory 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 DiseaseDiagnostic Criteria

1-Recurrent oral ulceration (at least three times in one of 12-month period)

Plus two of 2-Recurrent genital ulceration3-Eye lesions4-Skin lesions5-Positive pathergy tes

Yields a sensitivity of 91% and a specificity of 96%

New Simple Way to Use Classification Tree for the Diagnosis of Behcet’s 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 2 “ Skin Manifestations 1 “

Behcet’s Disease 7 “

D.D

prognosis

Behcet DiseaseTreatment-Aphtous Lesions

Topical or interalesional steroid Oral corticosteroid Colchicine Dapsone Thalidomide MTX Cyclosporine-A Interferon-A

Behcet DiseaseTreatment-ocular Manifestation

Corticosteroid Cyclosporine-A Azathioprine Cyclophosphamide Chlorambucil Methotrexate Surgery

Behcet DiseaseTreatment- Vascular Manifestation

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