1. بسم الله الرحمن الرحيم

2. BEHCET’s DISEASE
Azami Ahad.MD.

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

4. 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

5. (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

6. 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.

7. Behcet’s Disease Pathogenesis

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

9. 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

10. Behcet’s Disease Oral Aphtous

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

13. Behcet’s Disease Genital Aphtous

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

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

16. Behcet’s Disease Acneiform nodules

17. Behcet’s Disease Pathergy Test (pustule Formation)

18. 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

19. Behcet’s Disease Hypopyon

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

21. Behcet’s Disease Vascular aneurysm

22. Behcet’s Disease Vascular Aneurysm

23. Behcet’s Disease Venous Thrombosis

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

25. Behcet’s Disease Stroke

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

27. 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)

28. 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%

29. 11th ICBD Antalya 2004 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 “
Genital Aphthosis 3 “
Pathergy Phenomenon 2 “
Skin Manifestations 1 “
Behcet’s Disease 7 “

30. D.D
prognosis

31. Behcet Disease Treatment-Aphtous Lesions
Topical or interalesional steroid
Oral corticosteroid
Colchicine
Dapsone
Thalidomide
MTX
Cyclosporine-A
Interferon-A

32. Behcet Disease Treatment-ocular Manifestation
Corticosteroid
Cyclosporine-A
Azathioprine
Cyclophosphamide
Chlorambucil
Methotrexate
Surgery

33. Behcet Disease Treatment- Vascular Manifestation
Aspirin
Warfarin?
Corticosteroid
Azathioprine
Cyclosporine-A
Surgery: arterial aneurysm