TAKAYASU’S

ARTERITIS
Heba Maher Kamaly
DEFINITION
:TAKAYASU’S ARTERITIS
it is an inflammatory and stenotic disease of medium
. and large sized arteries
TA particularly affects the aorta and the pulmonary
Artery and the major arteries that arise from the aorta
(the subclavian , renal arteries ,coronary arteries and
.carotid arteries )
:It is called
AORTIC ARCH SYNDROME

.PULSELESS DISEASE
:EPIDEMIOLOGY
Uncommon disease.

Age:
Most patients are aged 5 - 53 years,
<15% of cases present in individuals older than 40 years.

Sex:
80% of patients are women.
Annual incidence rate 1-3 cases/million.
 Race:
Takayasu arteritis is observed more frequently in patients of Asian or
Indian .
Japanese have a higher incidence of aortic arch involvement.
:Pathlogy
, Panarteritis
,inflammmatory mononuclear infiltrates
.giant cells

, Marked intimal proliferation

fibrosis ,scarring and vascularisation of
.media

.disruption,degeneration of elastic lamina

Narrowing of lumen +/- thrombus

Clinical Presentation
.of patients with Takayasu arteritis are asymptomatic 10%

:Constitutional symptoms include the following

Headache (50-70%)
Malaise (35-65%)
Arthralgias (28-75%)
Fever (9-35%)
Weight loss (10-18%)
:Cardiac and vascular features include the following

Bruit, with the most common location being the carotid

artery (80%)
 Blood pressure difference of extremities (45%-69%)
Claudication (38-81%)
Carotodynia or vessel tenderness (pain and tenderness
over the carotid arteries in the front of the neck) ( 25
%).
Hypertension (28- 58%) with renal artery stenosis.
Aortic regurgitation (20-24%)
Raynaud’s syndrome (15%)
Pericarditis (<8%)
Congestive heart failure (<7%)
Myocardial infarction (<3%)
Neurologic features :

• Headache (50-70%)
• Visual disturbance (16-35%) - Strong association with
common carotid and vertebral artery disease
• Stroke (5-9%)
• Transient ischemic attacks (3-7%)
• Seizures (0-20%)
Dermatologic manifestations :

• Erythema nodosum (6-19%)

• Ulcerated subacute nodular lesions (<2.5%)
:PHYSICAL EXAMINATION
 Absent or diminished pulses are the clinical
hallmark of Takayasu arteritis, but pulses are
normal in many patients and upper limbs are affected
more Than lower limbs.

blood pressure in all 4 extremities:

The most finding is a systolic blood pressure
difference (>10 mm Hg) between arms.
 Hypertension due to renal artery involvement is
found in 50% of patients.
Carotidynia may be present.
Bruits are often noted ( loud noise indicating
narrowed arteries)
Aortic regurgitation is a common finding.
Ophthalmologic examination: may show retinal
hemorrhages,
cotton-wool exudates,
venous dilatation and beading,
microaneurysms of peripheral retina,
optic atrophy,
vitreous hemorrhage.
Skin changes resembling erythema nodosum or
ulcerating nodular lesions may be seen.
DIFFERENTIAL DIAGNOSIS
AORTIC COARCTATION
ATHEROSCLEROSIS
BEHCET’S DISEASE
BUERGER’S DISEASE (THROMBANGITIS
OBLITERANS)
GIANT CELL ARTERITIS
KAWASAKI DISEASE
RHEUMATOID ARTHRITIS
SARCOIDOSIS
 Classification criteria
The American College of Rheumatology has established classification
criteria for Takayasu arteritis (3 of 6 criteria are necessary). The presence
of any 3 or more criteria has a sensitivity of 90% and a specificity of
98%.
The criteria are as follows:
1. Age of 40 years or younger at disease onset.
2. Claudication of the extremities(severe muscle pain due to cramp,
caused by narrowed blood vessels).
3. Decreased pulsation of one or both brachial arteries.
4. Difference of at least 10 mm Hg in systolic blood pressure between
arms.
5. Bruit over one or both subclavian arteries or the abdominal aorta.
6. Arteriographic narrowing or occlusion of the entire aorta,or its primary
branches, or large arteries in the upper or lower extremities that is not
due toz other causes.
LABORATORY STUDIES
Blood Tests:
There is no blood test that can be used to
definitively confirm the diagnosis.

 The erythrocyte sedimentation rate (ESR) and C-

reactive protein (CRP) can help in measure activity of
the disease and response to treatment.
Investigation :

Complete blood count (CBC)

 Electrocardiogram (ECG)
Arteriogram
Angiogram
Magnetic resonance angiography (MRA)
Magnetic resonance imaging (MRI)
Ultrasound
X-ray of the chest
1. (MRI) Magnetic resonance
imaging is commonly used to aid
diagnosis and assess the
extent of TA.
•MRI is a safe means to monitor
the progress of
disease
and response to
treatment.
•The picture of the arteries can be
enhanced by using a contrast
medium .

. The illustration
shows an MRI scan of a TA patient
2. Ultrasound - Doppler ultrasound
(ultrasound scan):
This allows assessment
of blood
flow and can detect and
monitor the arterial wall and
the degree of narrowing.

Color Doppler is a useful tool for screening

and follow-up, particularly for carotid and
subclavian arteries.

This illustration shows a normal carotid

artery and one from a TA patient. The arrows
show the
thickening of the artery wall causing the
narrowing of the artery.
3. Positron emission tomography (PET
scanning): the patient be injected with radioactive fluorodeoxyglucose.
PET scans are particularly good at detecting inflammation in the
arterial wall and are often used in combination with CT scanning (CT-PET).
PET may identify early active disease and may help to distinguish active
disease from scarring wich is resulting from previous inflammation.
The illustration shows two PET images from the same patient; the one on the left is
before treatment, with the arrows pointing to
areas of inflammation. These areas have disappeared in the image on the right
which was taken after six months treatment.
5. Computed tomography (CT) angiography:
Contrast-enhanced CT angiography is useful for
the
diagnosis of TA and can help identify the extent of
damage to the arteries.
6. Angiography:

While (CT scanning, MRI) show typical patterns of

stenosis or aneurysms of the arteries, angiography
remains the standard for diagnosis and evaluation of
the extent of disease.
 recent studies suggest that noninvasive imaging such as
MRI, ultrasonography,and 18F-fluorodeoxyglucose
positron emission tomography (18F-FDG-PET) allow
diagnosis of Takayasu arteritis earlier in the disease
course than standard angiography and provide monitoring
of disease activity.
Angiography is used to evaluate only the appearance
of the lumen and cannot be used to differentiate
between active and inactive lesions
7. MRA ( Magnetic resonance angiography ) : is
more sensitive than angiography for lesions in the aorta .
Takayasu arteritis divided into 6 types based on
angiographic involvement :

• Type I - Branches of the aortic arch

• Type IIa - Ascending aorta, aortic arch, and its branches
• Type IIb - Type IIa region plus thoracic descending aorta
• Type III - Thoracic descending aorta, abdominal aorta,
renal arteries, or a combination
• Type IV - Abdominal aorta, renal arteries, or both
• Type V - Entire aorta and its branches
Assessing disease activity
worsening of 2 or more of the following features indicates
active disease :
1. Systemic features, such as fever and arthralgia (no
identified cause)
2. Elevated (ESR) erythrocyte sedimentation rate.
Features of vascular ischemia or inflammation, such as .3
claudication, diminished or absent pulse, bruit, carotodynia, or
asymmetrical blood pressure in the upper or lower limbs (or
.both)
.Typical angiographic feature .4
TREATMENT
Treatment is aimed TO improving symptoms and
preventing further damage to the blood vessels.
When there is active inflammation in the arteries, treatment
is started with steroids, usually prednisolone.
 Corticosteroids are the main of therapy for active Takayasu
arteritis, and some patients may require additional cytotoxic
agents to achieve remission and decrease chronic corticosteroid
treatment.
Oral corticosteroids are started at 1 mg/kg daily or divided twice
daily and tapered over weeks to months as symptoms subside.
Long-term low-dose corticosteroid therapy may be required.
Cytotoxic agents are used for patients whose disease is
steroid-resistant or relapsing.
Those commonly used include methotrexate, azathioprine .
 These drugs increase the effectiveness of steroid therapy
and help to reduce the dose of steroids required.

 These agents are usually continued for at least one year after
remission and are then tapered to discontinuation

AS:
Methotrexate - 7.5-25 mg/wk oral
Azathioprine - 1-2 mg/kg/d oral
In patients with persistent or very severe disease,
cyclophosphamide may be recommended
Cyclophosphamide - 2 mg/kg/d oral .
In recent years, for refractory disease, biologic
therapies, including tumour necrosis factor inhibitors
such as etanercept and infliximab have been used
successfully, as has tocilizumab which targets
interleukin-6.

ANTI –TNF AGENTS:

was effective in patients with active, relapsing Takayasu
arteritis despite treatment with steroids and multiple other
.immunosuppressive agents
 The initial dose of etanercept was 25 mg twice weekly,
 infliximab was given at 3 mg/kg initially and at 2 weeks, 6
weeks, and every 8 weeks thereafter.
Additional treatment:
 Drugs to lower blood pressure are often
recommended.
 Statins, the most commonly prescribed to lower
cholesterol and reduce the risk of heart disease. In TA
they may also have a beneficial effect on arterial
function, even when cholesterol levels are normal.
 Strict management of traditional cardiovascular risk
factors such as dyslipidemia, hypertension, and lifestyle
factors is mandatory to minimize secondary
cardiovascular complications, which are the major cause
of death in this disease.
 low-dose aspirin may have a therapeutic effect in large
vessel vasculitis and to reduce the risk of a blood clot
developing.
Surgery: Surgical intervention is only indicated for
the most severe cases of TA.
 For optimal results surgery should be carried out when the
disease is in remission.
Procedures that may be carried out include:
1. Revascularisation - where a blocked artery is bypassed,
typically with a vein graft from somewhere in the body.
Good long-term outcomes have been seen, with 20 year graft
survival rates >70 per cent in one study.
2. Percutaneous transluminal angioplasty (PTA) - aims to
open narrowed arteries by inserting and inflating a
balloon to stretch the narrowed section. In some cases a wire
mesh (stent) may be required to keep the artery open.
:PROGNOSIS
 The long term prognosis of TA is good.
 Approximately 20 per cent of patients will have a monophasic self-
limiting disease (just one inflammatory episode).
 More typically, the disease follows a relapsing and remitting course.
Limited data is available on long-term outcome and varies significantly
between patients, dependent on the extent of their disease.
 25 per cent of patients largely unaffected by their disease
 25 per cent of patients able to perform normal daily activities when
disease in remission
 50 per cent were unable to maintain full their full range of daily
activities
 Up to 25 per cent of patients are unable to work due to their Takayasu’s
arteritis
MORTALITY/MORBIDITY
Takayasu arteritis is a chronic relapsing and remitting
disorder.

10-year survival rate is approximately 90%; however, this

rate is reduced in the presence of major complications.
Complications
 valvular heart disease,
 stroke,
 heart failure,
 retinopathy,
 renovascular hypertension.

 The 5- and 10-year survival rates are approximately 69% in patients with
two cpmplications and 36%, in more than 2 complications. The 5- and 10-
year survival rates associated with one complications are 100% .
Pregnancy and Takayasu arteritis

Many women with Takayasu’s arteritis achieve one or more

successful pregnancies.
 Depending on how advanced disease is and how much
damage has been done to arteries,
 TA itself will not affect fertility.
some of the drugs used to treat the condition may reduce fertility
other medications may increase the risk of defects in the foetus.

it may be dangerous for a woman:

 For example control of blood pressure can be difficult in patients
with TA and blood pressure can be increased in pregnancy to a
dangerous level.