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Pediatric Takayasu Arteritis Clinical Presentation

  • Author: Christine Hom, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Nov 03, 2015
 

History

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)
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Physical Examination

The following symptoms may be observed on physical examination:

  • Blood pressure difference greater than 30 mm Hg between arms [12]
  • 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[13] :

  • 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.[14]

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Contributor Information and Disclosures
Author

Christine Hom, MD Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College

Christine Hom, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association, Arthritis Foundation

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

Thomas JA Lehman, MD, FAAP, FACR Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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Aortogram of a 15-year-old adolescent girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery.
MRI of thorax of 15-year-old adolescent girl with Takayasu arteritis. Note aneurysms of descending aorta.
Coronal MRI of abdomen of 15-year-old adolescent girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys.
 
 
 
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