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Takayasu’s Arteritis

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Overview

Takayasu’s arteritis is a rare autoimmune inflammatory disorder of unknown aetiology affecting the aorta and its main branches. It predominantly affects young women.

The vascular inflammation can cause stenosis, aneurysm, and occlusion of these arteries. 

Epidemiology

  • More common in Asia, specifically Japan
  • Affects women more frequently than men
  • Most common ages affected are 15-20 years

Risk Factors & Associations

  • Family history
  • Female sex
  • Asian ethnicity
  • Age <40 years

Presentation

The classical presentation of Takayasu’s arteritis is a young, female patient with systemic upset (fever and malaise) with unequal blood pressures in the upper limbs.

The presentation of Takayasu’s arteritis often presents in two stages, the first being the systemic stage:

  • Fever, fatigue, weight loss
  • Arthralgia and non-specific pains
  • Tenderness over the sites of the affected arteries

The occlusive stage then follows, whose features present depending on the site of ischaemia secondary to occlusion:

  • Cardiac:
    • Angina
    • Congestive cardiac failure
  • Pulmonary
  • Vascular
    • Jaw claudication
    • Claudication of the extremities
    • Back pain due to the involvement of the aorta
    • Hypertension
      • This is the most common presentation in children
  • Gastrointestinal
    • Abdominal pain due to bowel ischaemia/infarction
  • Renal
  • Dermatological
    • Erythema multiforme
  • Neurological

Signs on Examination

  • Systolic blood pressure difference of >10mmHg between the arms
  • Peripheral pulses may be weak or absent
    • A good way of remembering this sign is the pun “can’t Takaya pulse”
  • High blood pressures due to renal artery involvement
  • Arterial bruits
  • Anaemia
  • Muscle wasting

Differential Diagnoses

Giant cell arteritis

  • Patients are usually significantly older
  • Jaw claudication is common
  • Lower extremity involvement is less common

Essential hypertension

  • Pulses are intact on either side
  • No significant differences in blood pressures on either side
  • No bruits present

Tuberculosis

  • Persistent productive cough
  • Recent travel to a high-risk area

Kawasaki disease

  • Typically affects children <5 years
  • High-grade fever present
  • Lymphadenopathy present
  • Conjunctivitis present
  • Rash and peeling of the skin may be present
  • Strawberry tongue may be seen

Marfan’s syndrome

  • Patients are tall with long limbs
  • May have a family history of Marfan’s syndrome
  • Systemic symptoms are usually absent

Ehlers-Danlos syndrome

  • May have hypermobile joints
  • Systemic symptoms absent

Investigations

  • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP):
    • Usually elevated in active disease
  • CT angiography (CTA) – required for diagnosis:
    • Shows narrowing/occlusion of affected vessels
    • Aortic aneurysms may be seen
    • Thickening of blood vessel walls may be seen
  • Magnetic resonance angiography (MRA):
    • Shows soft tissue as well as arterial walls
    • Can identify active and inactive disease

Management

  • 1st line: glucocorticoids on a tapering regimen + low-dose aspirin + bone protection
  • If control is not achieved, then immunosuppressive therapy may be considered

Complications

  • Hypertension due to renal artery stenosis
  • Peripheral vascular ischaemia
  • Corticosteroid-related adverse effects
  • Aortic aneurysm
  • Aortic regurgitation
  • Congestive cardiac failure
  • Angina
  • Stroke
  • Loss of vision

Prognosis

  • 1/5 of patients have a self-limiting monophasic disease
  • In absence of major complications, the prognosis is excellent
  • Death is usually in untreated cases, most commonly due to heart failure or stroke as a result of hypertension secondary to renal artery stenosis

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