Transcript Document

Vascular involvement and
clinical criteria
in Behcet`s disease
Presented by:
Sasan Fallahi, MD
Rheumatologist, Kerman University of
Medical Sciences
History
• First description: Hippocrates in 5th century
before BC
• First modern: Hulusi Behcet in 1937
(a case with recurrent oral aphtous, genital aphtous, uveitis)
Epidemiology
• worldwide
• Most prevalent in silk road
( middle east, Mediterranean,…)
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Turkey: 80-370 in 100000
Iran: 80 in 100000
Japan: 13.6, China: 14 in 100000
Spain: 7.5 in 100000
Uncommon in northern Europe & USA
(0.1-7.5 in 100000)
Clinical manifestations
• Major:
* mucosal
* Skin
* eye
• Minor:
* joint, CNS, GI, vascular,…
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
Pathophysiology of vasculitis and thrombosis
TNFα, β,
INFɣ
Tissue
Superoxide,
NO
Antigen
(HSP)
IL1
Genetic background
Super Ag
B51
(APC)
T cell ( ɣδ )
PMN
CAM, IL8
Factor V Leiden
mutation
Macrophage
Vessel
wall
thrombosis
AECA
B cell
Hyperhomocysteinemia, antiphospholipid
Ab, decrease protein C, S, antithrombin ΙΙΙ
Pathogenesis of aneurisms
• Unclear
• Suggested:
Exogenous
factor
Genetic
background
Mononuclear
cells
Destruction of media, arterial
wall thickening
Aneurysm
formation
Functional
impairment
Obliterative endarteritis of
vasa vasorum, endothelial
cell swelling, perivascular
infiltration
Superficial thrombophlebitis
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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
DVT
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Males > females
More common in younger age of disease onset
Reported as an initial symptom
Critical period for development: 2-3.2 years after
diagnosis of Behcet disease
• Tend to occur earlier than arterial involvement
IVC thrombosis
• 15% of large vessel involvement
• Should be suspected in:
* alternating venous thrombosis in lower limbs
* Recurrent venous thrombosis in one limb
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
SVC thrombosis
• 1.4-9.8% of venous involvement
• Blockage of lymphatic circulation: chylothorax
Cerebral venous thrombosis
• Main symptom: persistent headache and
papilledema due to intracranial HTN, may be the
presenting feature of disease
• Is not rare
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%
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
Laboratory
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%
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)
ISG criteria(1990)
Sensitivity: 91%, specificity:96%
Iran criteria(1993)
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Oral aphthae
Skin lesions (PF, EN)
Pathergy test
Genital aphthae
Eye lesions
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1
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2
2
>= 3 score indicates Behcet disease
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
international criteria (ICBD)
Oral aphthosis
1
Skin lesions (PF, EN)
1
Vascular lesions
1
Pathergy test
1
Genital aphthosis
2
Eye lesions
2
3 or more points » Behcet’s Disease
(sensitivity: 96%, specificity: 88%, accuracy: 93.8%)
New international criteria (new ICBD)
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Oral aphthae
Skin lesions (PF, EN)
Vascular lesions
Genital aphthae
Eye lesions
CNS lesions
Pathergy test
2
1
1
2
2
1
1 (some where)
>= 4 score: sensitivity: 94%, specificity: 90.5%
(International society for Behcet disease, 8-10 July 2010, London)
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)
Morbidity:
• most common: ocular
blindness
Mortality:
• Low
• Related to pulmonary, CNS, bowel perforation