1. Behcet’s Syndrome N.Movaffagh MD Rheumatologist

2. DEFINITION
Behçet’s syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement
The diagnosis is clinical and based on internationally agreed diagnostic criteria

3. INCIDENCE Males and females are affected equally
Males often have more severe disease
Blacks are very infrequently affected
young males and females from the Mediterranean region, the Middle East, and the Far East, suggesting a link with the ancient Silk Route

4. PATHOGENESIS
The disease appears to be in the crossroads of autoinflammatory and autoimmune disorders.
The main pathologic lesion is : systemic perivasculitis with early neutrophil infiltration and endothelial swelling
Vasculitis of vasa vasorum(pseudoaneurysms)

5. PATHOGENESIS TH1, TH17, cytotoxic CD8+, and γδ T cells
Circulating autoantibodies against:
α-enolase of endothelial cells
selenium binding protein
Saccharomyces cerevisiae
Their pathogenic role remains unclear.

6. PATHOGENESIS
HLA-B*51
(MHC) class I
(IL) 10 locus

7. CLINICAL FEATURES recurrent aphthous ulcerations genital ulcers
Skin involvement
Eye involvement
arthritis or arthralgias
Superficial or deep peripheral vein thrombosis
Pulmonary emboli

8. Arterial involvement
Neurologic involvement
Gastrointestinal involvement
Epididymitis
amyloidosis of AA type and glomerulonephritis

9. Diagnostic Criteria of Behcet’s Syndrome
Recurrent oral ulceration plus two of the following:
Recurrent genital ulceration
Eye lesions
Skin lesions
Pathergy test

10. Oral aphthous ulcerations
Painful
shallow or deep
central yellowish necrotic base
singly or in crops
less than 10 mm(85%)
large or herpetiform lesions are less frequent
ulcers persist for 1–2 weeks
Subside without leaving scars

12. Genital ulcers less common More specific Painful
Do not affect the glans penis or urethra
produce scrotal scars

13. Skin involvement observed in 80% Folliculitis Erythema nodosum
Acne-like exanthema
vasculitis
Sweet syndrome
pyoderma gangrenosum

14. Erythema nodosum

15. pathergy test
Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection
a common and specific manifestation

16. Eye involvement in 50% of patients bilateral panuveitis with scarring
is the most dreaded complication
iritis, posterior uveitis
retinal vessel occlusions
optic neuritis
Blindness
usually present at the onset

17. Joint Involvement Nondeforming arthritis or arthralgias
in 50% of patients
knees and ankles

18. Superficial or deep peripheral vein thrombosis is seen in 30%
Pulmonary emboli are rare
The superior vena cava is obstructed occasionally

19. Arterial involvement in less than 5%
Aortitis or peripheral arterial aneurysm
Arterial thrombosis
Pulmonary artery vasculitis
(Dyspnea, cough, chest pain, hemoptysis, and infiltrates on chestX-ray)

20. Neurologic involvement
mainly in the parenchymal form (80%)
brainstem involvement
( CNS-Behcet’s syndrome)
IL-6 is persistently raised in csf

21. Cerebral venous thrombosis is most frequently observed in the superior sagittal and transverse sinuses headache and increased intracranial pressure
MRI,MRS are very sensitive

22. Gastrointestinal involvement
more frequently in patients from Japan
consists of mucosal ulcerations of the gut
Resembling Crohn’s disease

23. Epididymitis is seen in 5% of patients
amyloidosis of AA type and glomerulonephritis are uncommon

24. Laboratory findings
Leukocytosis
ESR
C-reactive protein

25. TREATMENT life expectancy seems to be normal
(Apart from the patients with CNS-Behcet’s syndrome and major vessel disease)
only serious complication is blindness

26. TREATMENT Mucous membrane involvement:
Topical glucocorticoids(mouthwash or past)
Thalidomide (100 mg/d)
Colchicine

27. Colchicine can be beneficial for the arthritis

28. TREATMENT
Thrombophlebitis is treated with aspirin, 325 mg/d

29. TREATMENT Uveitis and CNS-Behcet’s syndrome :
systemic glucocorticoid therapy (prednisone, 1mg/kg per day) and azathioprine (2–3 mg/kg per day)

30. TREATMENT sight-threatening uveitis :
Cyclosporine(5 mg/kg) alone or in combination with azathioprine

31. TREATMENT panuveitis refractory to immunosuppresives:
Anti–tumor necrosis factor

32. TREATMENT pulmonary or peripheral arterial aneurysms
Pulse doses of cyclophosphamide

33. Thank you