Wind of Change in the Treatment of Childhood-Onset Takayasu Arteritis: a Systematic Review


CURRENT RHEUMATOLOGY REPORTS, vol.23, no.8, 2021 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 23 Issue: 8
  • Publication Date: 2021
  • Doi Number: 10.1007/s11926-021-01032-8
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Keywords: Childhood-onset Takayasu arteritis, Pediatric Takayasu, Treatment, Tumor necrosis factor-alpha inhibitors, Interleukin-6 inhibitors, TOCILIZUMAB TREATMENT, EXPERIENCE, CHILDREN, EFFICACY, CLASSIFICATION, INFLAMMATION, INVOLVEMENT, CRITERIA, SAFETY
  • Hacettepe University Affiliated: Yes


Purpose of Review We lack evidence-based data for the treatment of childhood-onset Takayasu arteritis (c-TA) since it is a rare disease in children. In this systematic literature review, we aimed to evaluate the treatment choices in c-TA patients and integrate our experience for the treatment of our patients in the recent years/in the biologic era. Recent Findings We reviewed 24 articles addressing treatments of 413 c-TA patients. Steroids were given to 352 patients (85.2%) as the main immunosuppressive therapy. Other immunosuppressive agents included methotrexate (37.3%), cyclophosphamide (24.5%), azathioprine (16.9%), and mycophenolate mofetil (7.9%). Besides, various biological agents were used, including tumor necrosis factor-alpha inhibitors in 70 of 107 c-TA patients (65.4%) and interleukin-6 inhibitors in 33 of them (30.8%). Biologics are increasingly used in our center as well. Even in severe patients, CYC is switched to either anti-TNF or antiIL6 once disease control is achieved. Recently, in addition to conventional immunosuppressants, biologics are increasingly used in c-TA. We have revised our treatment protocol to start with 1-3 doses of high-dose steroids and CYC, in a child with TA with types III-V involvement and high acute phase reactants; once clinical features subside and CRP normalizes, biologics should be started to replace CYC while decreasing the steroid dose.