"Vascular Involvement and Clinical Criteria in Behcet's Disease: A Presentation by Sasan Fallahi, MD"

Slide1Vascular involvement andclinical criteria in Behcet`s disease Presented by: Sasan Fallahi, MD Rheumatologist, Kerman University of Medical Sciences

Slide2History• First description: Hippocrates in 5 th  century before BC • First modern: Hulusi Behcet in 1937     (a case with recurrent oral aphtous, genital aphtous, uveitis)

Slide3Epidemiology• 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)

Slide5Clinical manifestations•  Major:   *  mucosal  * Skin  * eye •  Minor:   * joint, CNS, GI, vascular,…

Slide6Vascular 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

Slide7Pathophysiology of vasculitis and thrombosisAntigen Antigen (HSP) (HSP) Antigen Antigen (HSP) (HSP) Genetic background B51 Super Ag Macrophage (APC) T cell (  ɣ δ  ) IL1 TNF α ,  β , INF ɣ B cell AECA Vessel wall t hrombosis PMN CAM, IL8 Superoxide, NO Tissue Hyperhomocysteinemia, antiphospholipid Ab, decrease protein C, S, antithrombin  ΙΙΙ Factor V Leiden mutation

Slide8Pathogenesis of aneurisms• Unclear • Suggested:   Exogenous Exogenous factor factor Exogenous Exogenous factor factor Genetic background Mononuclear cells Mononuclear cells Mononuclear cells Mononuclear cells Functional impairment Functional impairment Functional impairment Functional impairment Obliterative  endarteritis  of vasa  vasorum,  endothelial cell  swelling,  perivascular infiltration Obliterative  endarteritis  of vasa  vasorum,  endothelial cell  swelling,  perivascular infiltration Obliterative  endarteritis  of vasa  vasorum,  endothelial cell  swelling,  perivascular infiltration Obliterative  endarteritis  of vasa  vasorum,  endothelial cell  swelling,  perivascular infiltration Destruction  of  media,  arterial wall  thickening Destruction  of  media,  arterial wall  thickening Destruction  of  media,  arterial wall  thickening Destruction  of  media,  arterial wall  thickening Aneurysm formation Aneurysm formation Aneurysm formation Aneurysm formation

Slide9Superficial 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

Slide10DVT• 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

Slide11IVC thrombosis• 15% of large vessel involvement • Should be suspected in:     * alternating venous thrombosis in lower limbs     * Recurrent venous thrombosis in one limb

Slide12Budd-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

Slide13SVC thrombosis• 1.4-9.8% of venous involvement • Blockage of lymphatic circulation: chylothorax

Slide14Cerebral venous thrombosis• Main symptom: persistent headache and papilledema due to intracranial HTN, may be the presenting feature of disease • Is not rare

Slide15Peripheral 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%

Slide16Pulmonary 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

Slide18LaboratoryESR, 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%

Slide19Diagnosis & 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)

Slide20Sensitivity: 91%, specificity:96%ISG criteria (1990)

Slide21Iran criteria(1993) • Oral aphthae                 1 • Skin lesions (PF, EN) 1 • Pathergy test                         1 • Genital aphthae 2 • Eye lesions 2 >= 3 score indicates Behcet disease

Slide22ITR-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

Slide23international 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%)

Slide24New 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)

Slide25•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

Slide26Morbidity:•  most common: ocular                blindness Mortality: • Low • Related to pulmonary, CNS, bowel perforation