TAKAYASU’S ARTERITIS

restriction

No race – immune

Incidence-2.6/million/year-N.America/Europe

The incidence in Asia is 1 case/1000-5000

women.

range from infancy to middle age
Indian studies-age 3- 50 yrs

Gender diff
Japan-F:M=8-9:1
India-F:M ratio varies from -1:1 - 3:1
( Padmavati S, Aurora AP, Kasliwal RR Aortoarteritis in India. J Assoc Physicians India 1987)
India=F:M- 6.4:1 (Panja et al, 1997 JACC)

Genetics

Japan - HLA-B52 and B39

Mexican and Colombian patients - HLA-DRB1*1301 and HLA-DRB1*1602

India- HLA- B 5, -B 21

&/ ectatic changes

Large vessels, esp, Aorta & its main branches

(brachiocephalic, carotid, SCL, vertebral, RA)
+Coronary & PA

Ao valve –usually not beyond IMA

Multiple segs with dis & skipped nl areas
or diffuse involvement

Gross
1)Gelatinous plaques-early
2)White plaques-collagen
3)Diffuse intimal thickening

Superficial– deep scarring

circumferential stenosis
4)Mural thrombus

5)2⁰ atheromatous changes
long standing,
HTN

Histology

Panarteritis-granulomatous lesion with giant cells
a/c phase diffuse infil-mono
granulomatous infil

2)c/c phase-coll rich fibrous tissue- adventitia thicker than media

3)Healed phase-no infl cells, vas media scarred

a/c inflammation → destroys arterial media → Aneurysm (fibrosis inadequate)

Stenotic

lesions predominate & tend to be B/L

Nearly all pts with aneurysms also have stenoses

Erthema multiforme
Bazins disease(eryt induratum)
churg strauss synd
reteroperitoneal fib
PAN,UC,CD etc

cervical, axillary lymphadenopathy

Disappear partly/ completely in 3 months

50% -no h/o

acute phase

Late ischemic phase

Sequel of occl of Ao arch/br


Diminished/absent pulses (84–96%)

Bruits (80–94%)

Hypertension (33–83% )

RAS(28–75%) &

CCF(28%)

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

no real visual

loss

Hypertensive retinopathy

Commonest
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

Mild -stage 1
Moderate -stage 2
Severe -stages 3 & 4

Flourescien angio sensitive

high frequency of lesions in the abdominal aorta, including the

renal arteries, leading to renovascular hypertension

HTN, AR, & Aneurysm

(event free survival -64%)
Single mild complication or no complication
5

year event free survival 97%
Single severe or multiple complications
5 year event free survival 59.7%
No deaths in groups I and IIA
19.6% mortality in groups IIB and III (CVA,CCF)

Subramanyan R, Joy J, Balakrishnan KG, et al.SCT. Natural
history of aortoarteritis (Takayasu’s arteritis). Circulation
1989; 80: 429-37.

1990

1995

for
Takayasu arteritis. Int J Cardiol 1996; 54 : S141-S147

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

Ao and PA after steroid therapy

within and surrounding inflamed vessels
vascular dilation
thickened aortic

valvular cusps
multifocal stenoses
concentric thickening of the aortic wall

Disadvantages
difficulty in visualizing small branch vessels and poor visualization of vascular calcification
may falsely accentuate the degree of vascular stenoses (renal & subclavian)

early phase - linear and continuous
late phase-patchy rather than continuous ,linear
shown to identify more affected vascular regions than morphologic imaging with MRI
does not provide any information about changes in the wall structure or luminal blood flow

sensitivities of 83% and specificity 100%
( Meller Jet al. Value of F-18 FDG hybrid camera PET and MRI in earlyTakayasu aortitis. Eur Radiol 2003)
Sensitivity of 92%, specificity of 100% and a diagnostic accuracy of 94%
( Webb M et al. The role of 18F-FDG PET in characterising disease activity in Takayasu arteritis. Eur J Nucl Med Imaging 2004

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

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- infliximab

disease is active

During Rx- regular clinical examination and ESR+ C-RP

initially - every few days

CT or MR angio - 3 to 12 months - (active phase of Rx), and annually thereafter

Criteria for active disease

continued –

of s.C-RP and 20 mm/h of ESR

or critical stenoses of ≥3 cerebral vessels
Moderate AR
Cardiac ischaemia with

confirmed coronary involvement
Aneurysms

Recommended at quiescent state-avoids compli
(restenosis, anastamotic failure, thrombosis, haemorrhage, & infection)

nature of TA
Diffuse nature of TA

segment lesions
Incomplete relief of stenoses
Dissection

stent

stenosis with pr grad 20mm,

nl-ESR
Tech success -28 lesions (85%) clin success-14(82%)
Failures - Coexistent abd Ao disease & tight, prox RAS
Tech diffi - tough, noncompliant stenoses, difficult to cross & resisted repeated, prolonged balloon inflations - backache & ↓SBP during balloon inflation
Follow-up –mean (8/12) -restenosis in 6 (21%)

Renal PTA in TA -tech difficulties; Short-term results - good, Complication rate-acceptable

Sharma s et al, AIIMS Am J Roentgenol. 1992 Feb;158(2):417-22

without any added risks
Balloon dilation diff from atherosclerotic lesions
Minimal

intimal involvement –permits easy wiring and balloon crossing
Resistance to dilation – high fibrotic element in the stenotic lesion
restenosis> frequent in TA - diffuse and long stenotic lesions

and stenting.

A in TA
24 pts →26 Scl A

VB insufficiency, UL claudication, or both
Aortography → (focal-14 ,< 3 cm,extensive-12)
Initial tech & clinical success – 81% (17 /19 steno,4/7occlu)
Follow-up → mean26 months → ISR -6 ( all ext)
Cumu patency –S/L-100/50%
Long-term results -excellent in focal lesions ,less durable extensive disease

Tyagi s et al, GB Pant Cardiovasc Intervent Radiol. 1998 May219-24

To compare PTA- Scl A in TA & athero
61 Scl A PTA (TA = 32 & athero = 23)
PTA succ in 52 stenotis,3 occl
TA -Higher balloon inflation P
TA -more residual stenosis
TA –restenosis more

restnosis could be effectively redilated

TA -Subclavian PTA - Safe, can be performed as effectively as in athero, good long-term results

pts (12+4)- HTN/severe b/l- LL claudication
Aortography – stenosis→ DTA-5, abd

Ao-10, Both -1
Initial tech & clinical success -100%
patency rate of 67% in a 52-month follow-up
Follow-up (mean 21months)- Restenosis -3
PTA has a definite role in TA management
residual gradient < 20 mm -criterion for successful aortoplasty
long-segment disease, dissection or persistence of a grad > 20 mm Hg after PTBA- aortic stenting

Rao AS et al, SCT  Radiology. 1993 Oct;189(1):173-9

not indicated
・IMA can’t be used often
occlu of Innomi A /

Scl A
calcification of aorta
High incidence of restenosis:36％
Angioplasty(PTCA)
・alternative to surgery
Very high incidence of restenosis:78％
DES-effectiveness ?

together with stenotic lesions
fusiform or saccular
one of the

major complications related to the prognosis in TA
Incidence of aneurysm rupture -low
Management - mainly surgical.
Covered stent-grafts may be useful