TAKAYASU

ARTERITIS
Josua
Rendy Aprilianus Jiwono

PEMBIMBING:
dr. Dicky Aligheri Wartono, Sp.BTKV(K)-VE
INTRODUCTION
• Takayasu arteritis is the most common chronic idiopathic
granulomatous inflammation of large arteries that produces a
potentially life-threatening vasculitis of the aorta and its major
branches.
• “Pulseless Disease”
• Has a varied geographical incidence, thought to be much more
common among Asian populations
• The highest ever prevalence of TA at 40 per million was estimated in
Japan and the lowest ever one at 0.9 per million in US
Da Cruz, E. M., Browne, L., Darst, J. R., Fonseca, B., & Jaggers, J. (2013). Takayasu Arteritis. Pediatric and
Congenital Cardiology, Cardiac Surgery and Intensive Care, 2239–2252
Chair M, et al. Takayasu Arteritis: A Diagnostic Approach. Indonesian Journal of Rheumatology. 2018:10(2).p1-5
 HISTORY
• Firstly described by Mikito Takayasu, a
Japanese ophthalmologist; at the 12th
Annual Meeting of the Japan
Ophthalmology Society held in 1908 in
Fukuoka
• 21‐year‐old woman whose eyegrounds
exhibited “coronary anastomosis,”
arteriovenous anastomosis around the
papilla.
• 1939 by Yasuzo Shinmi who used the
term ‘Takayasu’s Arteritis’ for the first
time
F. Numano. The story of Takayasu arteritis. Rheumatology 2002;41:103–106
 PATHOLOGY
• Takayasu arteritis affects large
arteries, mostly the aorta and its
branches, as a vasculitic process.
• Vessel wall inflammation leads to
concentric wall thickening,
fibrosis, and thrombus formation.
• Affected vessels may become
stenotic or develop aneurysms
and vascular remodeling

Vaideeswar P, Deshpande JR. Pathology of Takayasu arteritis: A brief review. Ann Pediatr Cardiol. 2013;6(1):52-
58. doi:10.4103/0974-2069.107235
PATHOGENESIS

Arnaud, L., Haroche, J., Mathian, A., Gorochov, G., & Amoura, Z. (2011). Pathogenesis of Takayasu’s arteritis: A
2011 update. Autoimmunity Reviews, 11(1), 61–67
PATOPHYSIOLOGY
1. Initial acute phase, also called the prepulseless or active
inflammatory stage of vasculitis
2. Chronic pulseless phase or sclerotic phase, the progressive
inflammatory process manifests through the formation of
granulomas composed of macrophages, epithelioid, and giant cells
3. Final stage, the pulseless end-stage sclerotic phase demonstrates
alterations in the arterial lumen and is characterized by stenosis
seen in 90–100%of patients

Da Cruz, E. M., Browne, L., Darst, J. R., Fonseca, B., & Jaggers, J. (2013). Takayasu Arteritis. Pediatric and
Congenital Cardiology, Cardiac Surgery and Intensive Care, 2239–2252
PATOPHYSIOLOGY
 DIAGNOSIS
CLINICAL
• Diagnosis may be delayed by years
• In Acute Phase : headaches, dizziness, fever, fatigue, weight loss,
myalgia, arthralgia, abdominal pain, nausea, cough,
lymphadenopathy, anemia, and transient skin rashes
• The most predominant symptom is hypertension (82.6 %), followed
by headaches (31 %), fever (29 %), dyspnea (23 %), weight loss (22 %),
vomiting (20.1 %), and abdominal pain (16.6 %)
• In Late Phase  presenting symptoms reflect chronicity with end-
organ ischemia, infarction, and stroke

De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
 DIAGNOSIS
SIGN & SYMPTOMS (ISHIKAWA’S CRITERIA)
• Cardinal limb sign or symptoms:
• Pulselessness
• Differences in pulses in the arms
• Unobtainable blood pressure
• Significant blood pressure differences in the arms
• Easy limb fatigability or pain
• Minor signs or symptoms
• Unexplained fever or high ESR (20 mm in the first hour, by the Westergren
method), or both
• Neck pain
• Transient amaurosis or blurred vision or syncope
• Dyspnea or palpitations or both, or hypertension or aortic regurgitation
De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
 DIAGNOSIS
ISHIKAWA’S DIAGNOSTIC CRITERIA (sensitivity was 84%)
• Presence of the obligatory criterion (Age <40 years) – 1 month
+
• Two major criteria
or
• One major criterion and two or more minor criteria
or
• Four or more minor criteria

De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
ISHIKAWA’S DIAGNOSTIC CRITERIA

De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
1990 ACR DIAGNOSTIC CRITERIA
• patient shall be said to
have TA if at least 3 of
these 6 criteria are
present.
• The presence of any 3 or
more criteria yields a
sensitivity of 90.5% and a
specificity of 97.8%.

De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
EULAR/PRINTO/PRES criteria
• The criteria for childhood TA (c-
TA) include angiographic
abnormalities in the aorta or its
main branches and pulmonary
arteries as a mandatory
criterion and five additional
features of c-TA
• c-TA is classified when the
patient presents the mandatory
criterion and at least one of the
five other features
• 100% of sensitivity and 99.9%
of specificity
De Souza, A. W. S., & de Carvalho, J. F. (2014). Diagnostic and classification criteria of Takayasu arteritis. Journal
of Autoimmunity, 48-49, 79–83
 DIFFERENTIAL
DIAGNOSIS

Watson, L., Brogan, P., Peart, I., Landes, C., Barnes,

N., & Cleary, G. (2014). Diagnosis and Assessment
of Disease Activity in Takayasu Arteritis: A
Childhood Case Illustrating the Challenge. Case
Reports in Rheumatology, 2014, 1–6
 NUMANO CLASSIFICATION

Soto, M. E.,
Meléndez-Ramírez,
G., Kimura-Hayama,
E., Meave-Gonzalez,
A., Achenbach, S.,
Herrera, M. C., …
Reyes, P. A. (2011).
Coronary CT
Angiography in
Takayasu Arteritis.
JACC: Cardiovascular
Imaging, 4(9), 958–
966
WORKUP
• Diagnosis and follow-up of Takayasu arteritis may require a
combination of vascular imaging modalities:
• Conventional angiography  the gold standard imaging technique but has
largely become outdated
• Magnetic resonance imaging (MRI) and magnetic resonance angiography
(MRA)
• CT angiography (CTA) reliable tool in non-invasively depicting both luminal
and mural lesions in the aorta and its main branches
• Echocardiography
• Doppler ultrasound

Da Cruz, E. M., Browne, L., Darst, J. R., Fonseca, B., & Jaggers, J. (2013). Takayasu Arteritis. Pediatric and
Congenital Cardiology, Cardiac Surgery and Intensive Care, 2239–2252
 ANGIOGRAPH
Y
Example
A 39-year-old Case
female with TA
• (a, b) Axial precontrast CT image shows
the concentric thickened high-
attenuation wall [small arrows in (a)] in
descending thoracic aorta; the wall
appears low-attenuation compared
with the lumen [arrow in (b)] on post-
contrast images.
• (c) Innominate (arrowhead) and left
common carotid arteries (large arrow)
are involved.
• (d) Multiplanar reformatted image
illustrates the concentric thickening
wall of the left common carotid artery Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis:
(small arrows); the lumen is narrowed. imaging spectrum at multidetector CT angiography. Br J Radiol.
2012;85(1020):e1282-e1292. doi:10.1259/bjr/25536451
 ANGIOGRAPH
Y
Example
A 34-year-old femaleCase
with TA
• (a–d) The transmural calcification in
aortic arch anddescending aorta
(white arrows) can be observed,
while a stenotic lesion (black
arrows) is also depicted in maximum
intensity projection
• (b) and curved planar reformation
• (c) images at the segment of
diaphragmatic aortic hiatus, which is
associated with a regional dissection
[white arrowheads in (c, d)] at the
level of ostium of coeliac trunk. Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis:
imaging spectrum at multidetector CT angiography. Br J Radiol.
2012;85(1020):e1282-e1292. doi:10.1259/bjr/25536451
 ANGIOGRAPH
Y
Example
A 39-year-old femaleCase
diagnosed with
Takayasu arteritis with a complaint of
malaise and headache for 2 years.
• (a) The ringshaped high attenuation
(arrow) in pre-contrast axial CT
image represents the thickened
wall of descending thoracic aorta.
• (b, c) The thickened wall shows a
double ring enhancement pattern
(arrows) in arterial (b) and venous
phases (c) images. The stenotic
lumen is also visualized.
Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis:
imaging spectrum at multidetector CT angiography. Br J Radiol.
2012;85(1020):e1282-e1292. doi:10.1259/bjr/25536451
 ANGIOGRAPH
Y
Example
A 34-year-old femaleCase
Takayasu
arteritis patient with malaise,
dizziness, visual disturbance and
arthralgia of the left limb.
• (a–d) Curved planar reformation
and (e) axial images exhibit the
involvement of innominate (open
arrows), bilateral subclavian
(arrowheads and curved arrow) and
common carotid arteries (open
arrowhead and arrows).
• (f) Volume-rendered reformatted
image shows the luminal stenosis Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis:
of the diseased arteries. imaging spectrum at multidetector CT angiography. Br J Radiol.
2012;85(1020):e1282-e1292. doi:10.1259/bjr/25536451
 ANGIOGRAPH
Y
Example
A 41-year-old Case
female patient
diagnosed with Takayasu arteritis
with a complaint of hypertension
for 1 year.
• (a, b) Bilateral renal (open
arrows) and superior mesenteric
arteries (arrow) stenosis are seen
on the maximum intensity
projection images.
• (c) Volume-rendered reformatted
image shows thestenotic lesion
at the origin of bilateral renal Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis:
arteries (open arrows). imaging spectrum at multidetector CT angiography. Br J Radiol.
2012;85(1020):e1282-e1292. doi:10.1259/bjr/25536451
 ANGIOGRAPH
Y
A 29-year-old female Takayasu arteritis
Example
patient Caseand
with syncope, dyspnoea,
shortness of breath for 4 months, and left
limb claudication for 3 months.
• (a, b) The mildly narrowed distal segment of
left common carotid artery associated with
a severely stenotic lesion (arrows) at the Zhu FP, Luo S, Wang
original segment makes a ‘‘rat-tail’’-like ZJ, Jin ZY, Zhang LJ,
configuration in (a) curved planar Lu GM. Takayasu
reformation and (b) volume-rendered arteritis: imaging
images. spectrum at
multidetector CT
• (c) Maximum intensity projection and (d)
angiography. Br J
volume rendered reformatted images of the Radiol.
same patient illustrate the dilation of 2012;85(1020):e12
descending thoracic aorta associated with 82-e1292.
gradually narrowing distal segment (open doi:10.1259/bjr/25
arrows) and abdominal aorta, producing a 536451
MANAGEMENT

Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu
Arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365.
 MANAGEMENT

• Glucocorticoids  first line agents for patients with TA and begun at a

dose of 1 mg/kg per day for 1 to 3 months.
• Non Glucocorticoid  Methotrexate (MTX), Azathiprine (AZA), Tumor
Necrosis Factor Inhibitor (TNFi)
• If there is improvement in disease activity, the prednisone dose may
be tapered and ultimately discontinued.
• If there is no improvement consider switching to another non-GC
immunosupressive agent (MTX, AZA, TNFi, Tocilizumab (TCZ))

Ehlert BA, Abularrage CJ. Takayasu Disease. In: Sidawy AN, Perler BA, editors. Rutherford's vascular surgery and endovascular therapy 9th ed. Philadephia: Elsevier; 2019. p.1827-1839
 MANAGEMENT

Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365.
Ehlert BA, Abularrage CJ. Takayasu Disease. In: Sidawy AN, Perler BA, editors. Rutherford's vascular surgery and endovascular therapy 9th ed. Philadephia: Elsevier; 2019. p.1827-1839
 SURGICAL INTERVENTION
General indications for surgical revascularization
include
• hypertension in the setting of renal artery
stenosis
• life stylelimiting extremity ischemia
• cerebral ischemia or greater than 70%
symptomatic stenosis of cerebral vessels
• moderate (grade II New York Heart
Association) aortic regurgitation
• cardiac ischemia in the setting of proven
coronary artery stenosis
• severe aortic coarctation
• or progressive aneurysmal enlargement and
dissecting aneurysm.
Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365.
Ehlert BA, Abularrage CJ. Takayasu Disease. In: Sidawy AN, Perler BA, editors. Rutherford's vascular surgery and endovascular therapy 9th ed. Philadephia: Elsevier; 2019. p.1827-1839
OVERALL RESTENOSIS
STROKE
MORTALITY
THANK YOU