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Vascular involvement and clinical criteria in Behcet`s disease

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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


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