1 / 27

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 5 th century before BC First modern: Hulusi Behcet in 1937

keiki
Download Presentation

Vascular involvement and clinical criteria in Behcet`s disease

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Vascular involvement and clinical criteriain 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: 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)

  4. Clinical manifestations • Major: • * mucosal • * Skin • * eye • Minor: • * joint, CNS, GI, vascular,…

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

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

  11. 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

  12. SVC thrombosis • 1.4-9.8% of venous involvement • Blockage of lymphatic circulation: chylothorax

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

  14. 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%

  15. 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

  16. 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%

  17. 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)

  18. ISG criteria(1990) Sensitivity: 91%, specificity:96%

  19. 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

  20. 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

  21. 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%)

  22. 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 (somewhere) >= 4 score: sensitivity: 94%, specificity: 90.5% (International society for Behcet disease, 8-10 July 2010, London)

  23. 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)

  24. Morbidity: • most common: ocular blindness Mortality: • Low • Related to pulmonary, CNS, bowel perforation

More Related