1. BEHCET’S DISEASE
Hulûsi Behçet ( )

2. Main Distribution: Silk Root Male/Female Ratio
EPIDEMIOLOGY
Main Distribution: Silk Root
Male/Female Ratio
Variable
Near the same in large series
Mean Age:
Genetic Origin: Turkic Tribe

3. Silk Road

4. Prevalence/100,000 UK 0.64 Portugal 1.5 Germany 2.26 Japan 13.4 Turkey
80-370
Italia 2.5
Spain 7.5
China 14
Iran 16.7
Egypt 7.6
Saudi Arabia 20
Kuwait 1.2

5. Pathogenesis Unknown Genetic predisposition
Infection / environmental pollution
Immunological reaction
Damage of vascular endothelium

6. HLA-B51-associated Relative Risk for the occurrence of Behçet’s disease6 3 3 5 7 3 1 4 11 3 9 10 2 4 5 8 12 9 4 9 20 5 9 9 1

7. Triggering Agents Bacteria (Strep. oralis (sanguis) Viruse (HSV)
Chemicals (heavy metals)

8. Genetic predisposition
HLA-B51

9. Bacterial infection
Strep. oralis (sanguis) dominates the flora of the oral mucosa in patients with the disease.
A chronic infection with IgA protease-producing Strep. sanguis species is suggested.

10. Partial transcription of the HSV-I genome in patients´ peripheral blood mononuclear cells.
Identification of a 211-bp HSV-I DNA fragment in patients´ peripheral blood cells.
HSV-I DNA was detected in 43% of 91 saliva patients´ samples compared to 14% of 87 samples from healthy controls (p<0.01).
Viral infection

11. Immunological parameters
Strep. sanguis hsp-60 and -65 induce proliferation of gd+CD8+ T-cells.
MICA protein is recognised by T-cells with a variable Vd1gd region in their T-cell receptor.
Antigens are presented to gd+CD8+ T-cells assisted by the MICA molecule.
Immunological parameters

12. Behçet’s disease: a systemic disorder
Skin involvement
(Folliculitis/pustules, erythema nodosum, pyoderma, necrotising ulcers,
superficial thrombophlebitis, pathergy reaction)
Cerebral manifestations
(sterile meningoencephalitis,
vasa neurorum)
Uveitis
(Hypopyon-Iritis, Retinitis)
Oral aphthous ulcers
Cardiac involvement
(Pericarditis, endocarditis)
Lung involvement
(Aneurysma, embolic lesions,
haemorrhagy)
Kidney involvement
(Vasculitis)
Phlebothrombosis
Arterial aneurysma
Prostatitis
Gastrointestinal involvement
Genital ulcers
(Vasa vasorum)
(Gastritis, ulcers,
Pseudo-Crohn)
Arthritis

14. Clinical presentation
A common clinical feature in patients is painful, recurrent muco-cutaneous ulcers.
Oral ulcers:
Size ranges from a few mm to 2 cm.
Grossly and histologically similar to common oral ulcers. Multiple.
Often heal spontaneously in 1-3 weeks.

15. Genital ulcers Occur in 75% of patients.
Appear similar to oral ulcers.
Most frequently scrotal in men, vulvular in women.
Epididymitis, salpingitis, urethritis

16. Cutaneous lesions
Acneiform lesions, nodules, erythema nodosum, superficial thrombophlebitis, palpable purpura, pyoderma-gangrenosum.

18. Ocular disease
Uveitis is bilateral or unilateral, episodic sometimes with no resolution between episodes.
Hypopyon: purulent material present in anterior chamber indicating severe anterior uveitis.
Posterior uveitis, retinal vasculitis, vascular occlusion, optic neuritis: may progress to blindness if untreated. May require immunosuppressive treatment.
Glaucoma, cataracts can occur.

20. Neurological disease More common in males
Occurs in less than 20% of patients
Commonest abnormalities are vascular thrombosis and focal parenchymal lesions.
Manifestations include: progressive personality change, psychiatric disorders, dementia, aseptic meningitis, encephalitis. Peripheral neuropathy is not common.
Arteritis can lead to dissection, aneurysmal dilatation and subarachnoid hemorrhage.

21. Vascular disease
Venous involvement occurs more commonly than arterial.
Superficial and deep venous thrombosis is common.
Many patients have small vessel vasculitis.
30% have large vessel involvement, including stenosis and aneurysms.
Pulmonary arterial involvement is characteristic.

22. Arthritis Arthritis is nonerosive, inflammatory oligoarthritis
Occurs in 50% of patients
Asymmetric and usually does not result in deformities.
Commonly affects medium/large joints.
Occurs particularly during exacerbations, lasts 1-3 wks.

23. Diagnostic criteria Recurrent oral ulcers at least 3 times in a year
Recurrent genital ulcers
Eye inflammation
Skin inflammation
Pseudo folliculitis
Acne-like lesions
Erythema nodosum-like
Positive Pathergy test

24. DIFFFERENTIAL Reiter's syndrome and other forms of spondyloarthropathy
Inflammatory bowel disease (Crohn's disease and ulcerative colitis)
Syphilis
Erythema nodosum
Aphthous stomatitis

25. DIFFFERENTIAL Herpes simplex Stevens-Johnson syndrome Vasculitis
Multisystem disease
Thrombophlebitis related to coagulation factor deficiency

26. Prognosis and clinical course
Variable with exacerbations and remissions. Typical presentation is initially with mucocutaneous lesions and subsequently ocular and neurological disease occurring years after diagnosis.
Mortality is low and is usually related to pulmonary or CNS hemorrhage or bowel perforation.
Most common cause of morbidity is ocular disease which can result in blindness if untreated.

27. Treatment
Is based on degree of involvement and individual presentation.
Studies of treatment protocols are small.
Agents used- mucocutaneous disease
Topical steroids
Colchicine
Dapsone

28. Severe mucocutaneous disease
Methotrexate
Prednisone
Interferon Alpha
Thalidomide
Systemic disease
Azathrioprine
Cyclophosphamide
Anti-TNF drugs

29. Complications Death Blindness Paralysis
Embolism/thrombosis - pulmonary, vena cava, peripheral
Aneurysms
Amyloidosis
Thrombotic events, especially when anticardiolipin antibodies present

30. Normal life expectancy, except with neurologic involvement
Possible vision impairment
Avoid English Walnuts!!