Objective: Takayasu artery (TAK) is a chronic inflammatory disease that mainly affects the aorta and its major branches and is rarely reported in infants. We aimed to summarize the clinical features of infant TA (I-TA) in a tertiary care center. Methods: We performed a retrospective study involving 10 infants diagnosed with TAK. A comprehensive evaluation of clinical, laboratory, radiographic features, disease activity, treatment and outcomes was carried out. Results: A consecutive cohort was composed of 8 girls and 2 boys, with an age at diagnosis of 11.1 (1.7–36) months. The median time to diagnosis and the average time to follow-up were 9.5 days (2–235 days) and 10.9 (1–21) months, respectively. The most common initial manifestations were malaise (80%), fever (70%), hypertension (50%) and rash (30%). The mean Pediatric Vasculitis Activity Score (PVAS), Takayasu Clinical Activity Score (ITAS-2010) and ITAS-A scores were 2.8/63, 2.6/51, and 5.6/54, respectively. All patients had aberrant laboratory parameters. The most common lesions were in the thoracic aorta (60%) and abdominal aorta (60%). Corticosteroids combined with cyclophosphamide followed by long-term mycophenolate mofetil were initiated in most cases (70%). Biologics were attempted in 5 cases. Mortality was 40%. Conclusions: It is challenging to diagnose TAK in infants in a timely manner. Considering the more vessels involved, more severe inflammation and higher mortality, aggressive treatment is warranted in infants. GCs and CYC treatment seem to be effective.
CITATION STYLE
Jin, J., Zhao, Y., Gao, X., Wang, P., Liu, Y., Pan, Y., … Yu, H. (2024). A retrospective study of infantile-onset Takayasu arteritis: experience from a tertiary referral center in China. Frontiers in Cardiovascular Medicine, 11. https://doi.org/10.3389/fcvm.2024.1249305
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