Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis.
23 affiliations
- 1Department of Pediatrics, Stanford University, Palo Alto, California.
- 2British Columbia's Children's Hospital and the University of British Columbia, Vancouver, British Columbia, Canada.
- 3Nationwide Children's Hospital and The Ohio State University, Columbus.
- 4University of Chicago Medical Center, Chicago, Illinois.
- 5Department of Pediatrics, Medical College of Wisconsin, Milwaukee.
- 6University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom.
- 7Siriraj Hospital and Mahidol University, Bangkok, Thailand.
- 8Texas Children's Hospital and Baylor College of Medicine, Houston.
- 9Division of Allergy, Immunology, Rheumatology, Department of Pediatrics, University of Florida College of Medicine, Gainesville.
- 10Meyer Children's Hospital IRCCS, Florence, Italy.
- 11Children's Hospital of Pittsburgh and University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
- 12Birmingham Children's Hospital, Birmingham, United Kingdom.
- 13Primary Children's Hospital and University of Utah, Salt Lake City.
- 14Zagreb School of Medicine, Zagreb, Croatia.
- 15Pediatric Rheumatology, SRCC Children's Hospital, Mumbai, India.
- 16Royal Hospital for Children, Glasgow, United Kingdom.
- 17Pediatric Rheumatology, Western University and London Health Sciences Centre, London, Ontario, Canada.
- 18Department of Pediatrics, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
- 19Seattle Children's Hospital and Research Center, University of Washington, Seattle.
- 20Children's Hospital at Montefiore and the Albert Einstein College of Medicine, Bronx, New York.
- 21Riley Hospital for Children at Indiana University, Indianapolis.
- 22Alberta Children's Hospital and the University of Calgary, Calgary, Alberta, Canada.
- 23The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
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.
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BibTeX
@article{bistolarides2026clinical,
title = {{Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis.}},
author = {James Bistolarides and Kimberly A Morishita and Vidya Sivaraman and Linda Wagner-Weiner and Else S Bosman and Nick McPhate and James J Nocton and Melodee Liegl and Amy Y Pan and Michael W Beresford and Sirirat Charuvanij and Marietta de Guzman and Melissa Elder and Teresa Giani and Samuel Gagne and Katie Harrison and Karen James and Marija Jelusic and Archana Khan and Neil Martin and Jonathan Park and Alan M Rosenberg and Susan Shenoi and Tamara Tanner and Stacey E Tarvin and Marinka Twilt and Rae S M Yeung and Kelly Brown and David Cabral and ARCHiVe Investigators Network within the PedVas Initiative and the CARRA Chronic Childhood Vasculitis workgroup},
journal = {ACR open rheumatology},
year = {2026},
month = nov,
doi = {10.1002/acr2.90146},
url = {https://europepmc.org/article/MED/42971797},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis.
AU - James Bistolarides
AU - Kimberly A Morishita
AU - Vidya Sivaraman
AU - Linda Wagner-Weiner
AU - Else S Bosman
AU - Nick McPhate
AU - James J Nocton
AU - Melodee Liegl
AU - Amy Y Pan
AU - Michael W Beresford
AU - Sirirat Charuvanij
AU - Marietta de Guzman
AU - Melissa Elder
AU - Teresa Giani
AU - Samuel Gagne
AU - Katie Harrison
AU - Karen James
AU - Marija Jelusic
AU - Archana Khan
AU - Neil Martin
AU - Jonathan Park
AU - Alan M Rosenberg
AU - Susan Shenoi
AU - Tamara Tanner
AU - Stacey E Tarvin
AU - Marinka Twilt
AU - Rae S M Yeung
AU - Kelly Brown
AU - David Cabral
AU - ARCHiVe Investigators Network within the PedVas Initiative and the CARRA Chronic Childhood Vasculitis workgroup
PY - 2026
DA - 2026/11/01
JO - ACR open rheumatology
DO - 10.1002/acr2.90146
UR - https://europepmc.org/article/MED/42971797
AB - 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.
SN - 2578-5745
ER -