• Takayasu arteritis, also known as “pulseless disease, is a rare inflammatory disease primarily affecting the aorta and its major branches
  • predominantly occurs in young oriental women (female-to-male ratio 8:1)
  • etiology is unknown, but cell-mediated immune mechanisms are involved
  • histology is similar to temporal (giant cell) arteritis
    • inflammation begins with adventitial mononuclear infiltration
    • later, mononuclear inflammation of the media may occur, accompanied by granulomatous changes
  • onset typically in the first 3 decades of life
  • timely recognition and appropriate management are essential for improving patient outcomes in Takayasu arteritis cases
  • inflammation leads to irregular thickening of the affected artery, which may result in:
    • concentric stenosis or occlusion (initially with granulomatous changes of the media and adventitia, later involving intimal hyperplasia)
    • thrombosis
    • aneurysm formation (∼ 30% of patients)

Clinical presentation

Systemic symptoms

  • most common in the early stages
  • fatigue, fever, weight loss, night sweats, myalgia, and/or arthralgia
  • many patients may present only with focal symptoms due to hypoperfusion of the affected organ or limb

CNS involvement

  • symptoms of decreased cerebral perfusion
  • dizziness and syncope (> 50%)
  • subclavian steal syndrome
  • ischemic stroke
  • visual disturbances (visual field defects, amaurosis, retinal hemorrhages)
  • intracerebral hemorrhage (most commonly due to aneurysm rupture)

Involvement of the heart and other arteries

  • secondary renovascular hypertension
  • involvement of limb arteries
    • the diagnostic hallmark is a weakened pulse (“pulseless disease“); most patients have an absent pulse in at least one limb
    • decreased blood pressure in one or both arms
      • blood pressure difference > 20mm Hg between the left and right arm or between the arm and leg on the same side
    • murmurs are often audible over the subclavian arteries (in the supraclavicular fossa), brachial arteries, carotid arteries, abdominal aorta, or femoral arteries
    • limb claudication
    • Takayasu arteritis is a chronic disease in which collateral circulation usually develops; ischemic ulcerations or gangrene are uncommon
  • signs of aortic coarctation (e.g., hypertension, headache, leg claudication)
  • cardiac symptoms
    • angina pectoris / myocardial infarction (due to the narrowing of the coronary artery orifice)
    • heart failure due to aortic regurgitation
  • pulmonary hypertension, pulmonary infarcts

Diagnostic evaluation

The diagnosis of Takayasu arteritis is based on:

  • typical symptoms and signs (see above)
  • typical imaging findings
  • evidence of inflammation detected by blood tests

Imaging methods

  • characteristic findings in the aorta and its branches (always examine the descending aorta and renal arteries):
    • segmental concentric stenoses/occlusions
    • poststenotic dilatation
    • collateral arteries as a compensatory response to restricted blood flow
    • aneurysms
      • aortic aneurysms, in particular, require careful monitoring due to their potential for rupture
  • carotid ultrasound – allow direct visualization of wall thickening, may assess the blood flow in distal segments and collaterals
  • magnetic resonance imaging
    • MRA   Takayasu arteritis on MRI
    • T1C+ dark(black) blood sequence helps to detect inflammatory changes in the vessel wall   Takayasu arteritis - wall enhancement on contrast enhanced black blood sequences
  • CTA   Takayasu arteritis on CTA Takayasu arteritis on CTA
  • DSA  Takayasu arteritis (CCA) Takayasu arteritis (DSA)
  • Takayasu arteritis may progress silently even if clinical status and laboratory studies suggest remission ⇒  periodic vascular imaging is required
Takayasu on CTA

Takayasu arteritis - enlarged wall on ultrasound
Takayasu arteritis on ultrasound

Blood tests

  • tests are nonspecific
  • ↑CRP (C-reactive protein), ESR (erythrocyte sedimentation rate) > 40mm (however, normal values do not exclude active disease)
  • anemia of chronic disease (usually mild)
  • leukocytosis
  • elevated platelet count
  • ↑ metalloproteinases MMP-2, MMP-3 and MMP-9

Blood pressure measurement

  • BP should be measured in all extremities (both arms and both legs) to assess for potential discrepancies
  • accurate measurement can be challenging
    • if both subclavian arteries are affected, systemic BP can be measured only in the unaffected legs
    • if both subclavian arteries are affected, and the patient has also coarctation of the descending aorta, and/or involvement of both iliac/femoral arteries, accurate noninvasive BP measurement is impossible ⇒ central arterial pressure measurement via angiography may be required to detect occult hypertension
  • additional indicators of occult hypertension are:
    • hypertensive retinopathy
    • echocardiographic evidence of concentric left ventricular hypertrophy
  • severe hypertension may cause complications that may be mistaken for symptoms of vasculitis ⇒ a comprehensive evaluation and accurate diagnosis are essential

Diagnostic criteria and classification

Diagnostic criteria of Takayasu arteritis
American College of Rheumatology, 2022)
Absolute requirements
  • age ≤ 60 years
  • evidence of vasculitis in the aorta or its branches (by MR, CTA, DSA, ultrasound, PET)
Additional clinical criteria

  • female sex +1
  • angina or ischemic cardiac pain +2
  • arm or leg claudication +2
  • vascular bruit +2
    • detected by auscultation of large arteries
  • reduced or absent pulse in upper extremity +2
    • physical examination of the axillary, brachial, or radial arteries
  • carotid artery abnormity +2
    • reduced or absent pulse, tenderness of the carotid artery
  • SBP difference in arms ≥ 20 mmHg +1
Additional imaging criteria

  • number of territories involved (thoracic, abdominal aorta, mesenteric, left or right carotid, subclavian or renal artery)
    • one +1
    • two +2
    • three or more +3
  • symmetric involvement (stenosis, occlusion aneurysm) of paired arteries +1

    • subclavian, carotid, or renal arteries
  • abdominal aorta involvement with renal or mesenteric involvement +3
A score of ≥ 5 points is needed to classify as Takayasu arteritis
Diagnostic crieteria of Takayasu arteritis
American College of Rheumatology, 1990)
Typical clinical complaints lasting > 1 month
  • arm or leg claudication
  • decrease brachial artery pulse
  • BP difference > 10mmHg in both arms
  • amaurosis
  • syncope, palpitations
  • increased ESR
  • hypertension
  • aortic regurgitation
  • carotid artery tenderness
  Proof of arterial involvement

  • left subclavian artery
  • left subclavian artery
  • pulmonary artery
  • left CCA
  • brachiocephalic trunk
  • descending thoracic aorta
  • abdominal aorta
  • coronary arteries
  • annuloaortic ectasia (dilatation of the ascending aorta, the aortic annulus)
High probability of Takayasu arteritis

  • ≥ 2 major criteria
  • 1 major criterion + 2 minor criteria
  • 4 minor criteria

Differential diagnosis

  • atherosclerosis
    • age > 40 years
    • more common in men
    • lesions in typical locations
  • fibromuscular dysplasia (FMD)
    • rarely affects the subclavian artery; almost never the aorta
  • temporal arteritis (does not affect CCA and subclavian artery)
  • vascular infections
    • syphilitic aortitis (with typical aortic calcifications)
    • tuberculous, fungal aortitis
  • neurofibromatosis type I
  • idiopathic inflammatory conditions (e.g., ankylosing spondylitis with aortitis, Behçet syndrome, sarcoidosis)

Stroke prevention

  • antiplatelet therapy [Souza, 2010]
  • treatment of hypertension (problem with BP assessment in cases of subclavian artery stenosis/occlusion)
  • low sodium diet
  • stenting or bypass surgery in cervical artery lesion
    • procedures performed during remission have better outcome

Immunosuppressive therapy

  • start with PREDNISONE 1 mg/kg, then gradually taper over several weeks; target maintenance dose should be individualized (based on inflammatory markers and clinical status)
  • after remission is achieved (decrease in ESR and CRP, correction of anemia), maintain dose at 5-10 mg/day for 1-2 years
  • rapid tapering may lead to relapse
  • prevention of osteoporosis is necessary during long-term corticosteroid treatment – e.g., by administering Caltrate PLUS twice daily or Kombi-Kalz 1000/880 once daily
  • whenever possible, combine corticosteroids with immunosuppressants or immunomodulators to enhance treatment efficacy

Immunosuppressants – cytostatics

  • approximately 50-70% of patients require adjunctive treatment with immunosuppressive drugs
  • for less aggressive forms, consider the following:
    • azathioprine (IMURAN) 1-2 mg/kg/day
    • mycophenolate (CELLCEPT)
    • methotrexate is administered once weekly (due to its potential for toxicity)
      • starting dose: 7.5-15 mg once weekly
      • the dose may be gradually increased as needed and tolerated, with a usual maximum of ~ 20-25 mg per week
      • may help improve disease control and reduce corticosteroid dose
      • monitor patients for potential side effects, including hepatotoxicity, bone marrow suppression, and pulmonary toxicity
      • supplementation with folic acid is standard to mitigate some of the side effects
    • cyclophosphamide
      • typical dose 1-2 mg/kg per day
      • intravenous pulse of 500-1000 mg/m² body surface area, administered once a month

New immunosuppressive agents [Mekinian, 2015]

  • biologically targeted treatments
  • IL-6 receptor monoclonal antibodytocilizumab (ROACTEMRA)
    • used in the treatment of various autoimmune and inflammatory conditions
    • it has been investigated as an alternative or adjunctive therapy for Takayasu arteritis, especially in cases resistant to conventional treatment
    • results are promising but more research is needed to establish its long-term safety and efficacy
  • TNF-alpha blockers – reserved for conditions resistant to standard therapies

Reconstructive procedures

  • vascular procedures for organ-threatening ischemia, such as stenting or bypass surgery (indicated not only for cervical artery involvement but also for limb, mesenteric, and renal artery disease)

Follow up

Laboratory tests

  • CRP unreliable
  • ESR – decline during remission present in  ∼ 60% of cases ⇒ unreliable marker [Matsuyama,2003]
  • metalloproteinases [Matsuyama,2003]
    • MMP-2 diagnostic > 800 ng/mL
    • relapse – MMP-3 > 100 ng/mL and MMP-9 > 75 ng/mL

Imaging methods

  • vascular lesions may progress even if clinical and laboratory findings suggest remission
  • periodic vascular imaging to monitor stenosis grade and wall width/enhancement is essential
    • MRI of the neck  (with black blood sequences) + MRA
    • neurosonology for non-invasive flow dynamics evaluation
    • CT angiography


  • the clinical course is usually relapsing-remitting or chronic/progressive
  • despite therapy, the prognosis is uncertain and disease progression is frequently observed
  • despite combined immunosuppressive therapy, restenosis of intervened arterial segments is common

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Takayasu arteritis