Takayasu’s arteritis

Август 1, 2022

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Takayasu’s arteritis

Содержание

7. Epidemiology More case reports from Japan ,India, South-east Asia, Mexico No geographic restriction No race –
8. Age Mc-2nd & 3rd decade May range from infancy to middle age Indian studies-age 3- 50
9. Genetics Japan - HLA-B52 and B39 Mexican and Colombian patients - HLA-DRB1*1301 and HLA-DRB1*1602 India- HLA-
10. Histopathology Idiopathic inflammatory arteritis of elastic arteries resulting in occlusive/ ectatic changes Large vessels – Aorta
11. Pathogenesis Antigen-driven disease, with the site of immunologic recognition events being the adventitia. DC in adventitia
12. Pathological findings in Takayasu arteritis. Heather L. Gornik, and Mark A. Creager Circulation. 2008;117:3039-3051 Copyright ©
13. Macroscopic Gelatinous plaques-early White plaques-collagen Diffuse intimal thickening Superficial– deep scarring circumferential stenosis Mural thrombus 2⁰
14. Macroscopic Wall thickening, fibrosis, stenosis, thrombus formation →end organ ischemia More inflammation → destroys arterial media
15. Microscopic Panarteritis with inflammatory mononuclear cell infiltrates within the vessel wall with frequent giant cell formation
17. Clinical features Early pre-pulseless/gen manifestations Fever, weight loss,headache, fatigue,malaise,night sweats, arthralgia Splenomegaly, cervical, axillary lymphadenopathy Late
18. CLINICAL MANIFESTATIONS
20. Coronary involvement in TA Occurs in 10～30% Often fatal Classified into 3 types Type1:stenosis or occlusion
21. Occular involvement Hypertensive retinopathy Common Arteriosclerotic –art narrowing, av nipping,silver wiring Neuroretinopathy-exudates and papilloedema Direct opthalmoscopy
23. nee
24. Severe arteritis with complete occlusion of left carotid and subclavian artery. The right subclavian artery is
25. long-segment diffuse stenotic involvement of the DTA after deployment of stents.
26. remission after treatment
27. Figure 4. Takayasu arteritis involving the coronary ostia. Heather L. Gornik, and Mark A. Creager Circulation.
28. Figure 3. Aortic occlusive disease in a patient with Takayasu arteritis and bilateral leg claudication. Heather
29. Figure 7. Combination of 18F-FDG PET and CTA for assessment of Takayasu arteritis. Heather L. Gornik,
30. ostial stenosis of the right renal artery after deployment of a stent
31. a/c phase-Axial T1-weighted image wall thickening of As aorta and PA Axial T1-weighted image- improvement of
33. Diagnosis The diagnosis of Takayasu's arteritis should be suspected strongly in a young woman who develops
34. Treatment Disease-related mortality most often occurs from congestive heart failure, cerebrovascular events, myocardial infarction, aneurysm rupture,
35. Treatment of TA ・ Steroids immunosuppressants： Cyclosporine,Cyclophosphamide, Mtx,Mycophenolate mofetil Anti-platelet therapy（low-dose Aspirin） angioplasty/surgery If uncontrolled Control
36. Pharmacological treatment 0.7-1 mg/kg/day –prednisone for 1-3 months common tapering regimen once remission ↓ pred by
37. Steroids → 50% response Methotrexate →further 50% respond 25% with active disease will not respond to
38. Critical issue is in trying to determine whether or not disease is active During Rx- regular
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Epidemiology
More case reports from Japan ,India, South-east Asia, 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.

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Age
Mc-2nd & 3rd decade
May range from infancy 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

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Genetics
Japan - HLA-B52 and B39
Mexican and Colombian patients

- HLA-DRB1*1301 and HLA-DRB1*1602
India- HLA- B 5, -B 21

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Histopathology
Idiopathic inflammatory arteritis of elastic arteries resulting in 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

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Pathogenesis
Antigen-driven disease, with the site of immunologic 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

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Pathological findings in Takayasu arteritis.
Heather L. Gornik, and Mark A.

Creager Circulation. 2008;117:3039-3051

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Macroscopic
Gelatinous plaques-early
White plaques-collagen
Diffuse intimal thickening
Superficial– deep scarring
circumferential stenosis
Mural thrombus
2⁰

atheromatous changes
long standing,
HTN

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Macroscopic
Wall thickening, fibrosis, stenosis, thrombus formation →end organ ischemia
More inflammation →

destroys arterial media → Aneurysm (fibrosis inadequate)
Most patients with aneurysms also have stenosis

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Microscopic
Panarteritis with inflammatory mononuclear cell infiltrates within the vessel wall with

frequent giant cell formation
There is proliferation of the intima and fragmentation of the internal elastic lamina

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Clinical features
Early pre-pulseless/gen manifestations
Fever, weight loss,headache, fatigue,malaise,night sweats, 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%)

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CLINICAL MANIFESTATIONS

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Coronary involvement in TA
Occurs in 10～30%
Often fatal
Classified into 3 types
Type1:stenosis or

occlusion of coronary ostia
Type2:diffuse or focal coronary arteritis
Type3:coronary aneurysm

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Occular involvement
Hypertensive retinopathy
Common
Arteriosclerotic –art narrowing, av nipping,silver wiring
Neuroretinopathy-exudates 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

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Severe arteritis with complete occlusion of left carotid and subclavian artery.

The right subclavian artery is also occluded

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long-segment diffuse stenotic involvement of the DTA
after deployment of stents.

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remission after treatment

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Figure 4. Takayasu arteritis involving the coronary ostia.
Heather L. Gornik,

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

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Figure 3. Aortic occlusive disease in a patient with Takayasu arteritis

and bilateral leg claudication.

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

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Figure 7. Combination of 18F-FDG PET and CTA for assessment of

Takayasu arteritis.

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

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ostial stenosis of the right renal artery
after deployment of a stent

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a/c phase-Axial T1-weighted image
wall thickening of As aorta and

Axial T1-weighted image- improvement of wall thickening of As Ao and PA after steroid therapy

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Diagnosis
The diagnosis of Takayasu's arteritis should be suspected 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,

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Treatment
Disease-related mortality most often occurs from congestive 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

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Treatment of TA
　
・
Steroids
immunosuppressants：
Cyclosporine,Cyclophosphamide,
Mtx,Mycophenolate mofetil
Anti-platelet therapy（low-dose Aspirin）
angioplasty/surgery
If uncontrolled
Control of vasculitis
Symptomatic occlusion
thrombosis

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Pharmacological treatment
0.7-1 mg/kg/day –prednisone for 1-3 months
common 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

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Steroids → 50% response
Methotrexate →further 50% respond
25% with 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

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Critical issue is in trying to determine whether 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

Takayasu’s arteritis

Содержание

EpidemiologyMore case reports from Japan ,India, South-east Asia, MexicoNo geographic restrictionNo

AgeMc-2nd & 3rd decadeMay range from infancy to middle ageIndian

GeneticsJapan - HLA-B52 and B39 Mexican and Colombian patients

HistopathologyIdiopathic inflammatory arteritis of elastic arteries resulting in occlusive/ ectatic changesLarge

Pathogenesis Antigen-driven disease, with the site of immunologic recognition events being

Pathological findings in Takayasu arteritis. Heather L. Gornik, and Mark A.

MacroscopicGelatinous plaques-earlyWhite plaques-collagenDiffuse intimal thickening Superficial– deep scarring circumferential stenosisMural thrombus2⁰

MacroscopicWall thickening, fibrosis, stenosis, thrombus formation →end organ ischemiaMore inflammation →

MicroscopicPanarteritis with inflammatory mononuclear cell infiltrates within the vessel wall with

Clinical featuresEarly pre-pulseless/gen manifestationsFever, weight loss,headache, fatigue,malaise,night sweats, arthralgiaSplenomegaly, cervical, axillary

CLINICAL MANIFESTATIONS

Coronary involvement in TAOccurs in 10～30%Often fatalClassified into 3 typesType1:stenosis or

Occular involvementHypertensive retinopathyCommonArteriosclerotic –art narrowing, av nipping,silver wiringNeuroretinopathy-exudates and papilloedemaDirect opthalmoscopyNonhypertensive

nee