1. Vascular involvement and clinical criteria in Behcet`s disease
Presented by:
Sasan Fallahi, MD
Rheumatologist, Kerman University of Medical Sciences

2. History
First description: Hippocrates in 5th century before BC
First modern: Hulusi Behcet in 1937
(a case with recurrent oral aphtous, genital aphtous, uveitis)

3. Epidemiology
worldwide
Most prevalent in silk road
( middle east, Mediterranean,…)
Turkey: in
Iran: 80 in
Japan: 13.6, China: 14 in
Spain: 7.5 in
Uncommon in northern Europe & USA
( in )

4. 4

5. Clinical manifestations
Major:
* mucosal
* Skin
* eye
Minor:
* joint, CNS, GI, vascular,…

6. Vascular disorders Iran: 8.3%, Turkey: 17%, ITR-ICBD: 18% Any size
Venous: more common
*superficial phlebitis, DVT, large vein thrombosis
SVC, IVC, supra-hepatic, portal vein thrombosis
cerebral venous and retina thrombosis
Arterial:
*aneurism, thrombosis

7. Pathophysiology of vasculitis and thrombosis
TNFα, β, INFɣ
Tissue
Superoxide, NO
IL1
Genetic background
Antigen
(HSP)
Macrophage
(APC)
Super Ag
B51
T cell ( ɣδ )
PMN
Vessel wall
CAM, IL8
AECA
B cell
Hyperhomocysteinemia, antiphospholipid Ab, decrease protein C, S, antithrombin ΙΙΙ
Factor V Leiden mutation
thrombosis

8. Pathogenesis of aneurisms
Unclear
Suggested:
Exogenous
factor
Mononuclear cells
Functional impairment
Genetic background
Obliterative endarteritis of vasa vasorum, endothelial cell swelling, perivascular infiltration
Destruction of media, arterial wall thickening
Aneurysm formation

9. Superficial thrombophlebitis
Most frequent type
May predict visceral involvement
Increase risk of DVT
Main symptom: localized extremity pain
May occur after venipuncture
Females are affected more than males

10. DVT Males > females More common in younger age of disease onset
Reported as an initial symptom
Critical period for development: years after diagnosis of Behcet disease
Tend to occur earlier than arterial involvement

11. IVC thrombosis 15% of large vessel involvement Should be suspected in:
* alternating venous thrombosis in lower limbs
* Recurrent venous thrombosis in one limb

12. Budd-Chiari syndrome
Characterized by hepatomegaly, RUQ pain, ascites, oedema of lower limbs
Rarely, spontaneous remission
Mostly, at risk of slowly progressive hepatic failure,
Not common

13. SVC thrombosis 1.4-9.8% of venous involvement
Blockage of lymphatic circulation: chylothorax

14. Cerebral venous thrombosis
Main symptom: persistent headache and papilledema due to intracranial HTN, may be the presenting feature of disease
Is not rare

15. Peripheral artery involvement
Marked male predominance
Any peripheral artery: femoral, popliteal, iliac and abdominal aorta
Infrequent in carotid artery
Venous involvement is found in most patients
Occlusion is more common than aneurysm but both is not unusual finding
Clinical presentation: asymptomatic to pulsatile mass, back pain, painful mass, hematoma, intermittent claudication, abdominal pain, gangrene of forefoot, …
Rate of death: up to 60%

16. Pulmonary vascular involvement
The most sever: Pulmonary artery aneurism: leading cause of death
Almost exclusively in males
Most frequent symptom: chest pain, hemoptysis
Association with: DVT of lower limbs, vena cava or intra-cardiac thrombosis and aneurysms in other sites

18. Laboratory HLA B5: 53% in Iran HLA B51: 47% in Iran, ITR-ICBD: 49%
ESR, CRP (ESR in Iran: 40% normal)
HLA B5: 53% in Iran
HLA B51: 47% in Iran, ITR-ICBD: 49%
Pathergy: 54% in Iran, ITR-ICBD: 49%

19. Diagnosis & diagnostic criteria
Clinical
Diagnosis criteria:
-Curth, 1946
-Mason & barnes, 1969
-O Duffy: 1974
-Dilsen: 1986
-japan: 1972, 1988
-ISG: 1990
-Iran: 1993
- international criteria (ITR-ICBD) (2006)
- new ICBD (2010)

20. Sensitivity: 91%, specificity:96%
ISG criteria(1990)
Sensitivity: 91%, specificity:96%

21. Iran criteria(1993) Oral aphthae 1 Skin lesions (PF, EN) 1
Pathergy test
Genital aphthae 2
Eye lesions 2
>= 3 score indicates Behcet disease

22. ITR-ICBD (International Team for the Revision of the International Criteria for Behcet’s Disease)
Participants: 27 countries
Austria, Azerbaijan, China, Egypt, France, Germany, Greece, India, Iran, Iraq, Israel, Italy, Japan, Jordan, Libya, Morocco, Pakistan, Portugal, Russia, Saudi Arabia, Singapore, Spain, Taiwan, Thailand, Tunisia, Turkey, USA
Data Collection: March 2005 to June 2006
BD patients: 2556

23. international criteria (ICBD)
Oral aphthosis
Skin lesions (PF, EN) 1
Vascular lesions 1
Pathergy test
Genital aphthosis 2
Eye lesions 2
3 or more points » Behcet’s Disease
(sensitivity: 96%, specificity: 88%, accuracy: 93.8%)

24. New international criteria (new ICBD)
Oral aphthae
Skin lesions (PF, EN) 1
Vascular lesions 1
Genital aphthae 2
Eye lesions 2
CNS lesions
Pathergy test (some where)
>= 4 score: sensitivity: 94%, specificity: 90.5%
(International society for Behcet disease, 8-10 July 2010, London)

25. * arterial thrombosis: cytotoxic + prednisolon
Treatment
Vascular
* arterial thrombosis: cytotoxic + prednisolon
* DVT: cytotoxic + prednisolon
* superficial phlebitis: NSAID
* large vessel thrombosis: anticoagulant + cytotoxic + prednisolon
* Peripheral arterial aneurysm: arterioplasty + corticosteroid + cytotoxic
* Pulmonary arterial aneurysm: corticosteroid + cytotoxic ± embolization or surgery (in life threatening haemoptysis)

26. Morbidity: Mortality: most common: ocular blindness Low
Related to pulmonary, CNS, bowel perforation