학술논문
Predictive utility of ANCA positivity and antigen specificity in the assessment of kidney disease in paediatric-onset small vessel vasculitis
Document Type
article
Author
Dirk Foell; Adam Huber; Linda Wagner-Weiner; Susan Shenoi; Vidya Sivaraman; Sirirat Charuvanij; Jeffrey N Bone; David A Cabral; Paul Dancey; Susanne Benseler; Flora Mcerlane; Marek Böhm; Neil Martin; Phil Riley; Roberta Berard; Rae Yeung; Eslam Al-Abadi; Alan Rosenberg; Kamran Mahmood; Else S Bosman; Simranpreet K Mann; Kimberly A Morishita; Kelly L Brown; Melissa Elder; Stacey Tarvin; Kathryn Cook; Karen James
Source
RMD Open, Vol 10, Iss 2 (2024)
Subject
Language
English
ISSN
2056-5933
Abstract
Objectives 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.Methods 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.Results Overall disease activity at diagnosis was higher in PR3-ANCA and MPO-ANCA-seropositive individuals compared with ANCA-negative vasculitis. By 1 year, there were no significant differences, based on ANCA positivity or specificity, in the likelihood of achieving inactive disease (~68%), experiencing improvement (≥87%) 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 years after diagnosis. Relative to PR3-ANCA, MPO-ANCA seropositivity was associated with a higher likelihood of kidney involvement (OR 2.4, 95% CI 1.3 to 4.7, p=0.008) and severe kidney dysfunction (Kidney Disease Improving Global Outcomes (KDIGO) stages 4–5; OR 6.04, 95% CI 2.77 to 13.57, p