Pediatric Drugs

, Volume 21, Issue 2, pp 81–93 | Cite as

Childhood-Onset Takayasu Arteritis (c-TA): Current and Future Drug Therapy

  • Ruchika Goel
  • T. Sathish Kumar
  • Debashish DandaEmail author
Review Article


Childhood-onset Takayasu arteritis (c-TA) is the third most common systemic vasculitic disorder in children. Vascular stenosis is the main complication, and aneurysms are reported in 19–65% of cases, often in combination with stenotic lesions. Management of patients with c-TA is largely based on studies involving predominantly patients with adult-onset TA (a-TA). More widely used criteria for patients with c-TA have been devised by the joint European League Against Rheumatism, Pediatric Rheumatology International Trials Organization, and Pediatric Rheumatology European Society. Of the available imaging modalities, those that do not use radiation (color Doppler ultrasound and magnetic resonance angiogram) are preferred over 18F-labeled fluoro-2-deoxyglucose (18F-FDG) positron-emission tomography, computed tomography (CT), and CT angiogram in children. Remission rates have been reported to be lower in c-TA than in a-TA, and published mortality rates in c-TA range from 16 to 40%, which is much higher than reported in patients with a-TA. The usual drug therapy options include steroids plus steroid-sparing second-line immunosuppressants, such as mycophenolate, azathioprine, methotrexate, cyclophosphamide, and cyclosporine, along with antiplatelet agents. Interleukin-6 inhibitors such as tocilizumab, as well as the tumor necrosis factor inhibitors, are other aggressive therapeutic options. As yet, no randomized controlled trials have been conducted in c-TA.


Compliance with Ethical Standards

Conflict of interest

RG, TSK, and DD have no conflicts of interest that are directly relevant to the content of this article.


No sources of funding were used to conduct this study or prepare this manuscript.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Ruchika Goel
    • 1
  • T. Sathish Kumar
    • 2
  • Debashish Danda
    • 1
    Email author
  1. 1.Department of Clinical Immunology and RheumatologyChristian Medical CollegeVelloreIndia
  2. 2.Department of Child HealthChristian Medical CollegeVelloreIndia

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