Vascular involvement and clinical criteria in Behcet`s disease
description
Transcript of Vascular involvement and clinical criteria in Behcet`s disease
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,…)
• 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
AntigenAntigen(HSP)(HSP)AntigenAntigen(HSP)(HSP)
Genetic background
B51Super Ag Macrophage
(APC)
T cell ( ɣδ )
IL1
TNFα, β, INFɣ
B cellAECAVessel
wall
thrombosis
PMN
CAM, IL8
Superoxide, NO
Tissue
Hyperhomocysteinemia, antiphospholipid Ab, decrease protein C, S, antithrombin ΙΙΙFactor V Leiden
mutation
Pathogenesis of aneurisms
• Unclear• Suggested:
ExogenouExogenoussfactorfactor
ExogenouExogenoussfactorfactor
Genetic background
MononucleMononuclear cellsar cellsMononucleMononuclear cellsar cells
Functional Functional impairmenimpairmentt
Functional Functional impairmenimpairmentt
Obliterative Obliterative endarteritis of vasa endarteritis of vasa vasorum, endothelial vasorum, endothelial cell swelling, cell swelling, perivascular infiltrationperivascular infiltration
Obliterative Obliterative endarteritis of vasa endarteritis of vasa vasorum, endothelial vasorum, endothelial cell swelling, cell swelling, perivascular infiltrationperivascular infiltration
Destruction of media, Destruction of media, arterial wall thickeningarterial wall thickeningDestruction of media, Destruction of media, arterial wall thickeningarterial wall thickening
AneurysAneurysm m formationformation
AneurysAneurysm m formationformation
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
DVT
• 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)
Sensitivity: 91%, specificity:96%
ISG criteria(1990)
Iran criteria(1993)
• Oral aphthae 1
• Skin lesions (PF, EN) 1
• Pathergy test 1
• Genital aphthae 2
• Eye lesions 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 23 or more points » Behcet’s Disease
(sensitivity: 96%, specificity: 88%, accuracy: 93.8%)
New international criteria (new ICBD)
• Oral aphthae 2
• Skin lesions (PF, EN) 1
• Vascular lesions 1
• Genital aphthae 2
• Eye lesions 2
• CNS lesions 1• Pathergy test 1 (some where)
>= 4 score: sensitivity: 94%, specificity: 90.5%
(International society for Behcet disease, 8-10 July 2010, London)
• 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)
Treatment
Morbidity:• most common: ocular blindness
Mortality:• Low• Related to pulmonary, CNS, bowel perforation