Does Takayasu arteritis differ between children and adolescents? Insights from a Russian multicenter cohort

Takayasu arteritis (TA) is a chronic large-vessel vasculitis with significant age-related heterogeneity. Global data highlight differences between adult and pediatric TA, as well as among different pediatric age groups. However, the epidemiology and clinical features of childhood-onset TA in the Russian population are largely unknown.

To characterize the clinical and laboratory features, age-related differences, and long-term outcomes of TA in a Russian pediatric cohort.

A retrospective multicenter cohort study included 79 patients aged under 18 years with TA, diagnosed according to EULAR/PRINTO/PRES criteria, from four centers in the Russian Federation. Demographic, clinical, laboratory, and instrumental data were collected. Patients were stratified into two age groups according to WHO definitions: Children (aged less than 10 years, n = 25) and adolescents (aged 10 years to 17 years inclusive, n = 54). Disease activity was assessed using ITAS2010 and ITAS.A. Statistical analysis was performed using StatTech v.4.12.2.

The median age at onset was 11.0 (8.0-14.0) years, and the time before diagnosis was 8.0 (2.0-17.5) months; females were 78.5%. The most common clinical manifestations were malaise (84.8%), fever (54.4%), and vascular bruits (54.4%). Children under 10 years had a lower proportion of girls (P = 0.016), lower erythrocyte sedimentation rate, C-reactive protein, and platelet levels (P < 0.05), ITAS.A scores (8.9 ± 4.3 vs 12.0 ± 4.4, P = 0.005), required more surgical interventions at diagnosis (32% vs 7.4%, P = 0.008) compared to adolescents. At follow-up, 34.4% of patients had persistent vascular imaging activity despite normalized laboratory parameters. Biological drugs were used in 50.6% of patients. Three patients died, all due to postoperative complications.

This pioneer Russian cohort study of childhood-onset TA reveals a unique clinical phenotype. Younger children show lower levels of inflammation than adolescents, yet they still require more surgical intervention.