Pediatric Takayasu Arteritis Clinical Presentation

Updated: Jan 07, 2021
  • Author: Christine Hom, MD; Chief Editor: Lawrence K Jung, MD  more...
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Systemic symptoms in Takayasu arteritis include the following:

  • Fever, night sweats

  • Fatigue

  • Weight loss

  • Myalgia and/or arthralgia and/or arthritis

  • Skin rash (eg, erythema nodosum, pyoderma gangrenosum)

  • Headaches and/or dizziness and/or syncope

  • Congestive heart failure, palpitations, angina

  • Hypertension (may be paroxysmal)

Symptoms related to ischemia include the following:

  • Ischemic stroke and/or transient ischemic attack

  • Visual disturbances (eg, blurred vision, diplopia, amaurosis)

  • Carotidynia

  • Abdominal pain

  • Claudications (vary due to the development of collateral circulations; symptom is rare in children)


Physical Examination

The following symptoms may be observed on physical examination:

  • Blood pressure difference greater than 30 mm Hg between arms [13]

  • Asymmetrical pulses

  • Diminished or absent pulses - Midaortic lesions found in children may not affect pulses

  • Poststenotic dilatations producing what appear to be bounding pulses (often present)

  • Hypertension (may be paroxysmal) - Because this typically results from renovascular compromise, this is a high-renin hypertension

  • Bruits - Especially over subclavian arteries or the aorta

  • Skin lesions - Reported skin lesions include erythema nodosum–like lesions, pyodermagangrenosum, leukocytoclasticvasculitis, and panniculitis

Asymmetrical pulses (common) and absent pulses (rare) can be found even in the later stages of the disease (awareness of this is critical). Funduscopic examination may reveal the following:

  • Retinal hemorrhages

  • Cotton-wool exudates

  • Venous dilatation and beading

  • Microaneurysms of peripheral retina

  • Optic atrophy

  • Vitreous hemorrhage

  • Classic wreathlike, peripapillary arteriovenous anastomoses (extremely rare)

An international pediatric rheumatology consortium consisting of the European League Against Rheumatism, (EULAR), Pediatric Rheumatology International Trials Organization (PRINTO), and the Pediatric Rheumatology European Society (PReS) released the following classification criteria for Takayasu arteritis and other vasculitis syndromes in children in Ankara, Turkey in 2008 [14] :

  • Angiographic evidence of vasculitis (angiography, CT scanning, or MRI) and 1 of the 5 criteria below

  • Pulse deficit or claudication

  • Bruits

  • Four-limb blood pressure discrepancy

  • Hypertension

  • Acute phase reactant levels elevated

These criteria provide a sensitivity of 100% and specificity of 99% compared with 85% sensitivity for adult Takayasu criteria.

A retrospective review emphasized that constitutional symptoms coupled with objective findings of diminished pulses, bruits, and hypertension should raise clinical suspicion for Takayasu Arteritis in pediatric patients. The study also added that pharmacologic therapy alone can be successful in controlling disease progression, however surgery was successful in minimizing symptoms when medical therapies failed. [15]