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 5 th 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 TNFα, β, INFɣ Tissue Superoxide, NO Antigen (HSP) IL 1 Genetic background Super Ag B 51 (APC) T cell ( ɣδ ) PMN CAM, IL 8 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 • • • 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 B 5: 53% in Iran HLA B 51: 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) • • • Oral aphthae Skin lesions (PF, EN) Pathergy test Genital aphthae Eye lesions 1 1 1 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) • • 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
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