Презентация на тему Takayasu’s arteritis

Mexico
No geographic restriction
No race – immune
Incidence-2.6/million/year-N.America/Europe
The incidence in Asia

is 1 case/1000-5000 women.

to middle age
Indian studies-age 3- 50 y

Gender diff

Japan-F:M=8-9:1
India-F:M ratio varies from -1:1 - 3:1

Epidemiology

Genetics

Japan - HLA-B52 and B39
Mexican and Colombian patients - HLA-DRB1*1301 and HLA-DRB1*1602
India- HLA- B 5, -B 21

occlusive/ ectatic changes
Large vessels – Aorta and its main

branches (brachiocephalic, carotid, SCL, vertebral, RA)
Coronary and PA involvement
Aorta - usually not beyond IMA
Multiple segments with skipped areas
or diffuse involvement

recognition events being the adventitia.
DC in adventitia activated by

AG release IL-18 and chemokines that “recruit” T cells from vasa vasorum to the vessel wall
CD4+ T cells secrete interferon-γ→
stimulate macrophages and multinucleated giant cells
The results of this inflammatory cascade are :
granulomatous inflammation
destruction of the internal elastic lamina
arterial wall hyperplasia, smooth muscle cell proliferation, intimal thickening, vascular occlusion

and Mark A. Creager Circulation. 2008;117:3039-3051
Copyright © American Heart

Association, Inc. All rights reserved.

scarring

circumferential stenosis
Mural thrombus
2⁰

atheromatous changes
long standing,
HTN

ischemia
More inflammation → destroys arterial media → Aneurysm (fibrosis

inadequate)
Most patients with aneurysms also have stenosis

vessel wall with frequent giant cell formation
There is proliferation

of the intima and fragmentation of the internal elastic lamina

arthralgia
Splenomegaly, cervical, axillary lymphadenopathy
Late ischemic phase
Sequel

of occlusion of Ao arch/br
Diminished/absent pulses (84–96%)
Bruits (80–94%)
Hypertension (33–83% )
RAS(28–75%)

3 types

Type1:stenosis or occlusion of coronary ostia
Type2:diffuse or focal

coronary arteritis
Type3:coronary aneurysm

and papilloedema
Direct opthalmoscopy
Nonhypertensive retinopathy
UYAMA & ASAYAMA CLASS
stage 1- Dil

of small vessels
stage 2- Microaneurysm
stage 3- Art-ven anastomoses
stage 4- Ocular complications

and subclavian artery. The right subclavian artery is also

occluded

of stents.

Heather L. Gornik, and Mark A. Creager Circulation. 2008;117:3039-3051
Copyright

© American Heart Association, Inc. All rights reserved.

with Takayasu arteritis and bilateral leg claudication.
Heather L.

Gornik, and Mark A. Creager Circulation. 2008;117:3039-3051

Copyright © American Heart Association, Inc. All rights reserved.

for assessment of Takayasu arteritis.
Heather L. Gornik, and

Mark A. Creager Circulation. 2008;117:3039-3051

Copyright © American Heart Association, Inc. All rights reserved.

of a stent

As aorta and PA
Axial T1-weighted image- improvement of wall

thickening of As Ao and PA after steroid therapy

strongly in a young woman who develops a decrease

or absence of peripheral pulses, discrepancies in blood pressure, and arterial bruits.
The diagnosis is confirmed by the characteristic pattern on arteriography, which includes irregular vessel walls, stenosis, poststenotic dilation, aneurysm formation,

heart failure, cerebrovascular events, myocardial infarction, aneurysm rupture, or

renal failure.
The course of the disease is variable, and although spontaneous remissions may occur, Takayasu's arteritis is most often chronic and relapsing.
Glucocorticoid therapy for acute signs and symptoms.
An aggressive surgical and/or arterioplastic approach to stenosed vessels. Unless it is urgently required, surgical correction of stenosed arteries should be undertaken only when the vascular inflammatory process is well controlled with medical therapy.
In individuals who are refractory to or unable to taper glucocorticoids, methotrexate in doses up to 25 mg per week has yielded encouraging results.
Anti-TNF therapies have encouraging results

vasculitis
Symptomatic occlusion
thrombosis

tapering regimen once remission
↓ pred by 5 mg/week →

20 mg/day.
Thereafter, ↓by 2.5 mg/week → 10 mg/day
↓1 mg/day each week, as long as disease does not become more active
Pulse iv corticosteroids - CNS symptoms- no data to support

active disease will not respond to current treatments
resistant

to steroids/ recurrent disease once corticosteroids are tapered
cyclophosphamide (1-2 mg/kg/day),
azathioprine (1-2mg/kg/day), or
methotrexate (0.3 mg/kg/week)
Mycophenolate mofetil/ anti TNF α agents

or not disease is active
During Rx- regular clinical examination

and ESR+ CRP initially - every few days
CT or MRA - 3 to 12 months - (active phase of Rx), and annually thereafter
Criteria for active disease