{ "abstract": "Objective\n\nTo characterize the clinical features, treatment, and outcomes of a large, international cohort of childhood-onset Takayasu arteritis (cTAK).Methods\n\nPatients 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\n\nAmong 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\n\nAlthough 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.", "abstract_shown": true, "affiliations": [ "Department of Pediatrics, Stanford University, Palo Alto, California.", "British Columbia's Children's Hospital and the University of British Columbia, Vancouver, British Columbia, Canada.", "Nationwide Children's Hospital and The Ohio State University, Columbus.", "University of Chicago Medical Center, Chicago, Illinois.", "Department of Pediatrics, Medical College of Wisconsin, Milwaukee.", "University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom.", "Siriraj Hospital and Mahidol University, Bangkok, Thailand.", "Texas Children's Hospital and Baylor College of Medicine, Houston.", "Division of Allergy, Immunology, Rheumatology, Department of Pediatrics, University of Florida College of Medicine, Gainesville.", "Meyer Children's Hospital IRCCS, Florence, Italy.", "Children's Hospital of Pittsburgh and University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.", "Birmingham Children's Hospital, Birmingham, United Kingdom.", "Primary Children's Hospital and University of Utah, Salt Lake City.", "Zagreb School of Medicine, Zagreb, Croatia.", "Pediatric Rheumatology, SRCC Children's Hospital, Mumbai, India.", "Royal Hospital for Children, Glasgow, United Kingdom.", "Pediatric Rheumatology, Western University and London Health Sciences Centre, London, Ontario, Canada.", "Department of Pediatrics, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.", "Seattle Children's Hospital and Research Center, University of Washington, Seattle.", "Children's Hospital at Montefiore and the Albert Einstein College of Medicine, Bronx, New York.", "Riley Hospital for Children at Indiana University, Indianapolis.", "Alberta Children's Hospital and the University of Calgary, Calgary, Alberta, Canada.", "The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada." ], "article_type": "research article", "arxiv": null, "author_total": 30, "authors": [ { "affs": [ 0 ], "name": "James Bistolarides" }, { "affs": [ 1 ], "name": "Kimberly A Morishita" }, { "affs": [ 2 ], "name": "Vidya Sivaraman" }, { "affs": [ 3 ], "name": "Linda Wagner-Weiner" }, { "affs": [ 1 ], "name": "Else S Bosman" }, { "affs": [ 1 ], "name": "Nick McPhate" }, { "affs": [ 4 ], "name": "James J Nocton", "orcid": "0000-0003-3560-6099" }, { "affs": [ 4 ], "name": "Melodee Liegl" }, { "affs": [ 4 ], "name": "Amy Y Pan" }, { "affs": [ 5 ], "name": "Michael W Beresford" }, { "affs": [ 6 ], "name": "Sirirat Charuvanij" }, { "affs": [ 7 ], "name": "Marietta de Guzman" }, { "affs": [ 8 ], "name": "Melissa Elder" }, { "affs": [ 9 ], "name": "Teresa Giani" }, { "affs": [ 10 ], "name": "Samuel Gagne" }, { "affs": [ 11 ], "name": "Katie Harrison" }, { "affs": [ 12 ], "name": "Karen James" }, { "affs": [ 13 ], "name": "Marija Jelusic" }, { "affs": [ 14 ], "name": "Archana Khan", "orcid": "0000-0003-0842-2198" }, { "affs": [ 15 ], "name": "Neil Martin" }, { "affs": [ 16 ], "name": "Jonathan Park", "orcid": "0000-0001-6093-5500" }, { "affs": [ 17 ], "name": "Alan M Rosenberg" }, { "affs": [ 18 ], "name": "Susan Shenoi", "orcid": "0000-0002-2495-594X" }, { "affs": [ 19 ], "name": "Tamara Tanner" }, { "affs": [ 20 ], "name": "Stacey E Tarvin", "orcid": "0000-0001-8583-7878" }, { "affs": [ 21 ], "name": "Marinka Twilt", "orcid": "0000-0003-1954-2822" }, { "affs": [ 22 ], "name": "Rae S M Yeung", "orcid": "0000-0003-2361-725X" }, { "affs": [ 1 ], "name": "Kelly Brown", "orcid": "0000-0001-5385-3582" }, { "affs": [ 1 ], "name": "David Cabral", "orcid": "0000-0003-1840-9210" }, { "name": "ARCHiVe Investigators Network within the PedVas Initiative and the CARRA Chronic Childhood Vasculitis workgroup" } ], "bibtex": "https://research.draconitic.com/paper/54121rp818rvk.bib", "cited_by": 0, "doi": "10.1002/acr2.90146", "fulltext_url": "https://europepmc.org/articles/PMC13727717", "id": "54121rp818rvk", "issns": [ "2578-5745" ], "landing_url": "https://europepmc.org/article/MED/42971797", "language": "eng", "licence": "CC-BY-NC", "licence_label": "CC BY-NC", "licence_source": "article", "licence_url": "https://creativecommons.org/licenses/by-nc/4.0/", "metadata_licence": null, "native_subjects": [ [ "heading", "Original Article" ], [ "openalex", "2740|Vasculitis and related conditions" ] ], "openalex": "W7225094053", "pdf_url": "https://europepmc.org/articles/PMC13727717?pdf=render", "pmcid": "PMC13727717", "pmid": "42971797", "published": "2026-11-01", "publisher": "Wiley", "ris": "https://research.draconitic.com/paper/54121rp818rvk.ris", "sources": [ { "first_seen": "2026-11-04T15:20:44Z", "native_id": "MED:42971797", "source": "europepmc", "url": "https://europepmc.org/article/MED/42971797" }, { "first_seen": "2026-11-06T12:23:32Z", "native_id": "PMC13727717", "source": "pmc", "url": "https://doi.org/10.1002/acr2.90146" } ], "subjects": [ { "field": "medicine", "origin": "openalex:2740|Vasculitis and related conditions", "subfield": "general" } ], "title": "Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis.", "title_text": "Clinical Features, Treatments, and Outcomes of an International Cohort of 96 Children with Takayasu Arteritis.", "type": "peer-reviewed", "updated_at": "2026-11-07T12:20:51Z", "url": "https://research.draconitic.com/paper/54121rp818rvk", "venue": "ACR open rheumatology", "versions": [] }