Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis-a systematic review and meta-analysis

Clin Rheumatol. 2022 Dec;41(12):3601-3613. doi: 10.1007/s10067-022-06318-5. Epub 2022 Aug 5.


Takayasu arteritis (TAK) is a less common large-vessel vasculitis which can occur in either children or adults. However, differences between pediatric-onset and adult-onset TAK have not been systematically analyzed. We undertook a systematic review (pre-registered on PROSPERO, identifier CRD42022300238) to analyze differences in clinical presentation, angiographic involvement, treatments, and outcomes between pediatric-onset and adult-onset TAK. We searched PubMed (MEDLINE and PubMed Central), Scopus, major recent international rheumatology conference abstracts, Cochrane database, and, and identified seven studies of moderate to high quality comparing pediatric-onset and adult-onset TAK. Meta-analysis of 263 pediatric-onset and 981 adult-onset TAK suggested that constitutional features (fever, and in subgroup analyses, weight loss), hypertension, headache, and sinister features of cardiomyopathy, elevated serum creatinine, and abdominal pain were more frequent in pediatric-onset TAK, whereas pulse loss/pulse deficit and claudication (particularly upper limb claudication) were more frequent in adult-onset TAK. Hata's type IV TAK was more common in pediatric-onset TAK, and Hata's type I TAK in adult-onset TAK. Children with TAK also appeared to require more intense immunosuppression with more frequent use of cyclophosphamide, biologic DMARDs, tumor necrosis factor alpha inhibitors, and, in subgroup analyses, tocilizumab in pediatric-onset TAK than in adult-onset TAK. Surgical or endovascular procedures, remission, and risk of mortality were similar in both children and adults with TAK. No studies had compared patient-reported outcome measures between pediatric-onset and adult-onset TAK. Distinct clinical features and angiographic extent prevail between pediatric-onset and adult-onset TAK. Clinical outcomes in these subgroups require further study in multicentric cohorts.

Keywords: Aortitis syndrome; Childhood onset; Large-vessel vasculitis; Pediatric onset; Pediatric vasculitis; Takayasu arteritis.

Publication types

  • Meta-Analysis
  • Systematic Review
  • Review

MeSH terms

  • Adult
  • Antirheumatic Agents* / therapeutic use
  • Child
  • Cyclophosphamide / therapeutic use
  • Humans
  • Immunosuppression Therapy
  • Retrospective Studies
  • Takayasu Arteritis* / drug therapy
  • Takayasu Arteritis* / therapy


  • Cyclophosphamide
  • Antirheumatic Agents