Takayasu Arteritis 

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Overview

Takayasu arteritis is a rare, chronic large-vessel vasculitis primarily affecting the aorta and its major branches, leading to stenosis, occlusion, or aneurysm formation. It most commonly affects young women (<40 years), particularly of Asian descent. The global incidence is estimated at 1–2 per million per year, with a female predominance (F:M ~9:1). It is also called “pulseless disease” due to the hallmark feature of absent peripheral pulses in late-stage disease.

Takayasu arteritis is named after Mikito Takayasu, a professor of ophthalmology at Kanazawa University in Japan, who, in 1905, presented a case of a 21-year-old woman with wreath-like retinal vascular anastomoses at the Annual Meeting of the Japanese Ophthalmology Society.

Definition

Stenosis: Narrowing of a vessel, reducing blood flow.
Bruits: Audible vascular turbulence over narrowed vessels, a hallmark in TA.

Anatomy and Physiology

  • The aorta and its major branches (subclavian, carotid, renal arteries) are the primary vessels involved.
  • These are elastic arteries, accommodating high-pressure blood flow.

Remember

Subclavian and carotid artery involvement often explains neurological and upper limb symptoms in TA.

Aetiology and Risk Factors

Aetiology

  • Unknown; likely autoimmune-mediated granulomatous inflammation.
  • Possible links to infectious triggers (e.g., Mycobacterium tuberculosis).
  • Genetic associations (e.g., HLA-B52) reported in some populations.

Risk Factors

  • Female sex
  • Age <40 years
  • Asian or Latin American ethnicity
  • Family history (rare)

Pathophysiology

TAK is a panarteritis, but the initial site of inflammation is around the vasa vasorum and at the medio-adventitial junction. Large and medium-sized arteries are considered immune-privileged sites. Vascular dendritic cells, located near the vasa vasorum, function as gatekeepers by restricting lymphocyte entry and dampening local immune-inflammatory responses. 

  • Immune tolerance is disrupted when intrinsically abnormal dendritic cells (DCs) become activated by unknown stimuli. Abnormal DCs activate immune response towards large arteries.
  • Immune activation targets large arteries → granulomatous inflammation of vessel wall.
  • Inflammatory infiltrate damages elastic lamina and media → intimal hyperplasia and fibrosis.
  • Leads to:
    • Stenosis or occlusion of affected arteries
    • Aneurysm formation in weakened segments
    • Ischemia in affected organ systems (e.g., brain, limbs, kidneys)
  • Chronic phase dominated by fibrotic vessel wall thickening.

The phase of inflammation (active vs fibrotic) impacts treatment and imaging findings.

Clinical Manifestations

Takayasu arteritis most commonly presents in young women under 40–50 years of age, particularly of Asian descent.  Early Takayasu arteritis often goes undiagnosed because clinical features are nonspecific, but progression is associated with obstructive or aneurysmal lesions.

In addition to constitutional manifestation symptoms are related to the severity of specific arterial involvement.

PhaseDescription
Systemic (Pre-pulseless) phaseNon-specific inflammatory symptoms, mimicking infection or autoimmune disease
Occlusive (Vascular) phaseIschemic symptoms due to progressive arterial stenosis or occlusion
Artery InvolvedClinical Manifestations 
Subclavian arteryUpper limb claudication, asymmetric BP, subclavian steal syndrome
Carotid arteryAmaurosis fugax/Takayasu retinopathy (ophthalmic artery) TIA/stroke, carotidynia, cervical bruit
Vertebral arteryPosterior circulation TIA, vertigo, ataxia
Aortic arch/thoracic aortaPulse deficits, aortic regurgitation, thoracic bruit
Abdominal aortaMesenteric ischemia (intestinal angina), lower limb claudication, bruit
Renal arteriesRenovascular hypertension, hypertensive encephalopathy, CKD
Coronary arteriesAngina, myocardial infarction
Pulmonary arteriesPulmonary hypertension, dyspnea, hemoptysis

Suspect Takayasu in young female with unexplained hypertension, arm claudication, or pulse deficits.

Examination findings 

  • Unequal/absent pulses (usually upper limbs)
  • Blood pressure discrepancy between arms (>20 mmHg)
  • Bruits over subclavian/carotid/aorta
  • Hypertension (due to renal artery involvement)
  • Retinopathy (from carotid involvement)

Remember

Always check bilateral BP in young women with claudication or unexplained hypertension.

Diagnosis

Classification Criteria for Takayasu Arteritis (2022 ACR/EULAR)
CategoryFeaturePoints
Absolute RequirementsAge ≤ 60 years at time of diagnosisRequired
Evidence of vasculitis in the aorta or its branches on imagingRequired
Additional Clinical CriteriaFemale sex+1
Angina or ischemic cardiac pain+2
Arm or leg claudication+2
Vascular bruit over aorta or major arteries+2
Reduced or absent pulse in upper extremity (axillary, brachial, or radial arteries)+2
Carotid artery abnormality on imaging+2
Systolic blood pressure difference ≥ 20 mm Hg between arms+1
Additional Imaging CriteriaOne affected arterial territory+1
Two affected arterial territories+2
Three or more affected arterial territories+3
Symmetric involvement of paired arteries (e.g., bilateral carotid or renal arteries)+1
Abdominal aorta involvement with either renal or mesenteric arteries+3
Diagnosis = Total score ≥ 5

Investigations:

  • Inflammatory markers: ↑ ESR, ↑ CRP
  • Imaging:
    • CT Angiography (CTA) or MR Angiography (MRA): Vessel wall thickening, stenosis, aneurysm
    • PET-CT: Active inflammation
    • Conventional angiography: Gold standard for vessel lumen but not wall
  • Autoimmune panel: Usually negative (ANA, ANCA)

Differential Diagnoses:

ConditionDifferentiators
Giant cell arteritisAge >50, temporal artery involvement
AtherosclerosisOlder age, risk factors, calcifications
Fibromuscular dysplasiaString-of-beads appearance, no systemic symptoms
Coarctation of aortaCongenital, BP difference in upper vs lower limbs

Classification

Based on angiographic pattern (Numano classification):

TypeInvolvement
IAortic arch and branches
IIaAscending aorta, arch, branches
IIbIIa + thoracic descending aorta
IIIThoracic + abdominal aorta
IVAbdominal aorta, renal arteries
VEntire aorta and branches

Remember

Most patients have Type V, with widespread arterial involvement.

Treatment

PhaseTreatment
Active inflammationHigh-dose corticosteroids (prednisolone 1 mg/kg/day)
Steroid-sparingMethotrexate, azathioprine, mycophenolate mofetil
RefractoryBiologics (e.g. tocilizumab, TNF inhibitors)
Vascular complicationsRevascularization (angioplasty or bypass) after controlling inflammation

Monitoring: ESR/CRP, imaging every 6–12 months.

Treat inflammation aggressively before vascular repair to reduce restenosis.

Complications and Prognosis

Complications

  • Hypertension (renal artery stenosis)
  • Stroke, TIA
  • Aortic regurgitation or aneurysm
  • Limb ischemia
  • Retinopathy/visual loss
  • Treatment-related: Steroid toxicity, infection

Prognosis

  • Chronic relapsing course
  • 10-year survival >85% with treatment
  • Relapse rates 30–50%; require long-term follow-up

Poor Prognostic Factors

  • Delayed diagnosis
  • Extensive vascular involvement (Type V)
  • Severe hypertension
  • Cardiac or neurologic complications


References

  1. Johnston SL et al. Takayasu arteritis: a review. J Clin Pathol. 2002;55(7):481–486.
  2. Kerr GS et al. Takayasu arteritis. Ann Intern Med. 1994;120(11):919–929.
  3. Terao C et al. Genetic associations with Takayasu arteritis. Curr Opin Rheumatol. 2015;27(1):1–7.
  4. Maksimowicz-McKinnon K et al. Clinical features and prognosis of Takayasu arteritis. Arthritis Rheum. 2007;56(3):1000–1009.
  5. Hellmich B et al. EULAR recommendations for large vessel vasculitis. Ann Rheum Dis. 2020;79(1):19–30.

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