Background/Purpose: Takayasu’s arteritis is a chronic granulomatous disease that affects the vascular wall of the large arteries and can result in end organ damage. It is more prevalent in young women, but can also occur in the childhood. The disease is rare and there are few reports about the clinical features at this age. Our aim was to describe the clinical features of Takayasu’s arteritis in children and adolescents in our population.

Methods: In this Brazilian multicenter retrospective study including 10 pediatric rheumatology centers we identified 71 children and adolescents with Takayasu’s arteritis. Patients’ demographic, clinical, laboratory, angiographic, therapeutic data and disease outcome were recorded.

Results: Of the 71 patients, 51 (72%) were girls, with a mean age at onset of 9.2 years (range 4 months to 17.2 years); the average time to diagnosis was 1.2 years; and the mean follow-up time was 5.4 years., The most frequent angiographic type was type IV (41%) followed by type V (27%) and the abdominal aorta was the most affected vessel (63.4%). The main lesion was arterial stenosis (84.5% of patients). At initial presentation 80.6% of patients had increased acute phase reactants and 41% of patients had a positive Mantoux test. The predominant clinical symptoms at onset were constitutional (77.5%), followed by neurological (70.4%) and musculoskeletal symptoms (64.8%). The main cardiovascular manifestation was arterial hypertension (84.5%). At the final evaluation, neurological symptoms were predominant (22.7%) and decrease of peripheral pulses (66.7%) was the main cardiovascular manifestation. Sixty four (90.1%) patients were treated with corticosteroids, 30 patients (42.3%) with methotrexate as the first immunosuppressive treatment and 18 (25.4%) were treated with cyclophosphamide as initial therapy. Infliximab was used in only 4 patients throughout the follow-up. At the final evaluation, 55% of patients were in disease remission, 28% had active disease, 7% died and in 10% the outcome was unknown.

Conclusion: Takayasu’s arteritis is a rare childhood disease. In this multicenter study we observed a high rate of disease remission, however prospective studies are needed in order to better define overall disease outcome.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-brazilian-multicenter-study-of-71-children-and-adolescents-with-takayasus-arteritis/