{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T01:09:12Z","timestamp":1780448952976,"version":"3.54.1"},"reference-count":27,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2024,6,17]],"date-time":"2024-06-17T00:00:00Z","timestamp":1718582400000},"content-version":"unspecified","delay-in-days":16,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000245","name":"Michael Smith Health Research BC","doi-asserted-by":"publisher","award":["Scholar Award"],"award-info":[{"award-number":["Scholar Award"]}],"id":[{"id":"10.13039\/501100000245","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Arthritis Society Canada","award":["Ross Petty Arthritis Society Chair"],"award-info":[{"award-number":["Ross Petty Arthritis Society Chair"]}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["CGS-M"],"award-info":[{"award-number":["CGS-M"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["PJT-180302"],"award-info":[{"award-number":["PJT-180302"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["TR2-119188"],"award-info":[{"award-number":["TR2-119188"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100012285","name":"BC Children's Hospital","doi-asserted-by":"publisher","award":["Graduate Student Award"],"award-info":[{"award-number":["Graduate Student Award"]}],"id":[{"id":"10.13039\/100012285","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100012285","name":"BC Children's Hospital","doi-asserted-by":"publisher","award":["Salary Award"],"award-info":[{"award-number":["Salary Award"]}],"id":[{"id":"10.13039\/100012285","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2024,5,12]]},"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>The objective of this study is to evaluate whether anti-neutrophil cytoplasmic antibody (ANCA) seropositivity and antigen specificity at diagnosis have predictive utility in paediatric-onset small vessel vasculitis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Children and adolescents with small vessel vasculitis (n=406) stratified according to the absence (n=41) or presence of ANCA for myeloperoxidase (MPO) (n=129) and proteinase-3 (PR3) (n=236) were compared for overall and kidney-specific disease activity at diagnosis and outcomes between 1 and 2 years using retrospective clinical data from the ARChiVe\/Paediatric Vasculitis Initiative registry to fit generalised linear models.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Overall disease activity at diagnosis was higher in PR3-ANCA and MPO-ANCA-seropositive individuals compared with ANCA-negative vasculitis. By 1\u2009year, there were no significant differences, based on ANCA positivity or specificity, in the likelihood of achieving inactive disease (~68%), experiencing improvement (\u226587%) or acquiring damage (~58%). Similarly, and in contrast to adult-onset ANCA-associated vasculitis, there were no significant differences in the likelihood of having a relapse (~11%) between 1 and 2\u2009years after diagnosis. Relative to PR3-ANCA, MPO-ANCA seropositivity was associated with a higher likelihood of kidney involvement (OR 2.4, 95%\u2009CI 1.3 to 4.7, p=0.008) and severe kidney dysfunction (Kidney Disease Improving Global Outcomes (KDIGO) stages 4\u20135; OR 6.04, 95%\u2009CI 2.77 to 13.57, p&lt;0.001) at onset. Nonetheless, MPO-ANCA seropositive individuals were more likely to demonstrate improvement in kidney function (improved KDIGO category) within 1 year of diagnosis than PR3-ANCA seropositive individuals with similarly severe kidney disease at onset (p&lt;0.001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>The results of this study suggest important paediatric-specific differences in the predictive value of ANCA compared with adult patients that should be considered when making treatment decisions in this population.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/rmdopen-2024-004315","type":"journal-article","created":{"date-parts":[[2024,6,17]],"date-time":"2024-06-17T20:45:42Z","timestamp":1718657142000},"page":"e004315","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Predictive utility of ANCA positivity and antigen specificity in the assessment of kidney disease in paediatric-onset small vessel vasculitis"],"prefix":"10.1136","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-8086-3604","authenticated-orcid":false,"given":"Simranpreet K","family":"Mann","sequence":"first","affiliation":[{"name":"Department of Microbiology and Immunology, Faculty of Science, University of British Columbia, Vancouver, British Columbia, Canada"},{"name":"British Columbia Children's Hospital Research Institute, Vancouver, British Columbia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7704-1677","authenticated-orcid":false,"given":"Jeffrey N","family":"Bone","sequence":"additional","affiliation":[{"name":"British Columbia Children's Hospital Research Institute, Vancouver, British Columbia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Else S","family":"Bosman","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A","family":"Cabral","sequence":"additional","affiliation":[{"name":"British Columbia Children's Hospital Research Institute, Vancouver, British Columbia, Canada"},{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada"},{"name":"British Columbia Children's Hospital, Vancouver, British Columbia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberly A","family":"Morishita","sequence":"additional","affiliation":[{"name":"British Columbia Children's Hospital Research Institute, Vancouver, British Columbia, Canada"},{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of 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