Aeschlimann FA, et al. - The presenting features, course, and outcome of Takayasu arteritis (TAK) in pediatric population were analyzed in this study, along with comparing the efficacy of treatment regimens and exploring high-risk factors for adverse outcome. A high disease burden was reported in children suffering TAK; half of the children experienced flares and 7% died. Better control of disease activity was reported in association with biologic therapies.

Methods

• Researchers performed a single-center cohort study of consecutive children fulfilling the EULAR/PRINTO/PReS criteria for childhood TAK between 1986 and 2015.
• They documented clinical phenotypes, laboratory markers, imaging features, disease course and treatment.
• They assessed disease activity using the Pediatric Vasculitis Disease Activity Score at each visit.
• Disease flare defined as new symptoms and/or increased inflammatory markers necessitating therapy escalation and/or new angiographic lesions, or death was the study outcome.
• Logistic regression was performed to test relevant variables for flare and Kaplan-Meier analyses for comparing treatment regimens.

Results

• This study comprised a total of 27 children; 74% were female, median age at diagnosis was 12.4 years.
• Data reported that 22 (81%) children presented with active disease at diagnosis.
• The following treatment regimens were used: corticosteroids alone (15%), corticosteroids plus methotrexate (37%), cyclophosphamide (19%), or a biologic agent (11%).
• A total of 14 (52%) out of 27 children had adverse outcomes: 2 (7%) died within 6 months of diagnosis, and 13 (48%) experienced disease flares.
• In addition, researchers found that with biologic treatments, the 2-year flare-free survival was 80% compared to 43% in non-biologic therapies (p=0.03); at last follow-up, biologic therapies resulted in significantly higher rates of inactive disease (p=0.02).
• They identified no additional outcome predictor.