Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis

Objective To characterize the clinical features, treatment, and outcomes of a large, international cohort of childhood‐onset Takayasu arteritis (cTAK). Methods Patients with a diagnosis of cTAK were identified in A Registry of Childhood Vasculitis and divided into subcohorts by era‐of diagnosis: pre‐biologic (PB; 1986–2006), early biologic (EB; 2007–2017), and recently diagnosed (RD; 2018–present). Longitudinal patient data were collected at time of diagnosis (TOD), postinduction (PI; 3–6 months after TOD), and 12 months after TOD (12m). Meaningful clinical improvement was defined as at least a 50% reduction in the Pediatric Vasculitis Activity Score. Damage was assessed by the Pediatric Vasculitis Damage Index. Results Among 96 patients (69% female, median age 12.8 years [interquartile range 9.8–15.3]) from nine countries, 86% showed clinical improvement at PI and 90% maintained improvement at 12m. Glucocorticoids (GC; n = 84, 88%) and methotrexate (n = 47, 49%) were the two most common medications prescribed at TOD, often together and in combination with another medication. GC use continued at 12m in 79%. Patients more recently diagnosed were more likely to receive treatment with tumor necrosis factor inhibitors (0% PB, 26% EB, and 35% RD; P = 0.007) and reach 70% improvement (59% PB, 81% EB, and 100% RD; P = 0.007) at PI. However, neither improvement nor damage at 12m differed among the subcohorts. Conclusion Although treatment approaches for cTAK have evolved and most patients demonstrate sustained clinical improvement at 12m, these advances have not translated into substantial changes in outcomes, highlighting the need for optimized therapeutic strategies.

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ACR Open Rheumatology
Published
2026-11-01
DOI
https://doi.org/10.1002/acr2.90146
Primary Topic
Vasculitis and related conditions
Type
article
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article

Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis

Archana Khan, James J. Nocton, Kelly L. Brown, Jonathan Park et al.
ACR Open Rheumatology
Vasculitis and related conditions
article

Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis

Archana Khan, James J. Nocton, Kelly L. Brown, Jonathan Park, David A. Cabral, Else S. Bosman, Marija Jelušić, Vidya Sivaraman, Melissa E. Elder, Susan Shenoi, Rae S. M. Yeung, Michael William Beresford, Marinka Twilt, Linda Wagner‐Weiner, Kimberly A. Morishita, Katie Harrison, Amy Pan, Marietta De Guzman, Sirirat Charuvanij, Alan M Rosenberg, Stacey E. Tarvin, Samuel J Gagne, Tamara Tanner, Melodee Liegl, Nick McPhate, James Bistolarides, Teresa Giani, Karen James, for the ARCHiVe Investigators Network within the PedVas Initiative and the CARRA Chronic Childhood Vasculitis workgroup, Neil Martin
article en

Abstract

Objective To characterize the clinical features, treatment, and outcomes of a large, international cohort of childhood‐onset Takayasu arteritis (cTAK). Methods Patients with a diagnosis of cTAK were identified in A Registry of Childhood Vasculitis and divided into subcohorts by era‐of diagnosis: pre‐biologic (PB; 1986–2006), early biologic (EB; 2007–2017), and recently diagnosed (RD; 2018–present). Longitudinal patient data were collected at time of diagnosis (TOD), postinduction (PI; 3–6 months after TOD), and 12 months after TOD (12m). Meaningful clinical improvement was defined as at least a 50% reduction in the Pediatric Vasculitis Activity Score. Damage was assessed by the Pediatric Vasculitis Damage Index. Results Among 96 patients (69% female, median age 12.8 years [interquartile range 9.8–15.3]) from nine countries, 86% showed clinical improvement at PI and 90% maintained improvement at 12m. Glucocorticoids (GC; n = 84, 88%) and methotrexate (n = 47, 49%) were the two most common medications prescribed at TOD, often together and in combination with another medication. GC use continued at 12m in 79%. Patients more recently diagnosed were more likely to receive treatment with tumor necrosis factor inhibitors (0% PB, 26% EB, and 35% RD; P = 0.007) and reach 70% improvement (59% PB, 81% EB, and 100% RD; P = 0.007) at PI. However, neither improvement nor damage at 12m differed among the subcohorts. Conclusion Although treatment approaches for cTAK have evolved and most patients demonstrate sustained clinical improvement at 12m, these advances have not translated into substantial changes in outcomes, highlighting the need for optimized therapeutic strategies.

ACR Open RheumatologyVol. 8(11)
Western University (CA), Seattle Children's Hospital (US), Albert Einstein College of Medicine (US), Nationwide Children's Hospital (US), Primary Children's Hospital (US), Siriraj Hospital (TH), University of British Columbia (CA), University of Liverpool (GB), University of Calgary (CA), University of Zagreb (HR), Baylor College of Medicine (US), University of Toronto (CA), University of Washington (US), Medical College of Wisconsin (US), University of Utah (US), Mahidol University (TH), Royal Hospital for Children (GB), University of Florida Health (US), London Health Sciences Centre (CA), Hospital for Sick Children (CA), Alberta Children's Hospital (CA), Riley Hospital for Children (US), University of Saskatchewan (CA), University of Florida (US), University of Chicago Medical Center (US), Alder Hey Children's NHS Foundation Trust (GB), Birmingham Children's Hospital (GB), Meyer Children's Hospital (IT), Children's Hospital of Pittsburgh (US), University of Pittsburgh Medical Center (US), Children's Hospital at Montefiore (US), Texas Children's Hospital (US), BC Children's Hospital (CA), SRCC Children’s Hospital (IN), Alder Hey Children's Hospital (GB), The Ohio State University (US), Stanford University (US)
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Vasculitis and related conditions
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