Evaluating the utility of adult-defined biomarkers in childhood-onset chronic primary systemic vasculitis
department or school: Science, Faculty of
department or school: Microbiology and Immunology, Department of
data provider: DSpace
degree campus: UBCV
creator: Mann, Simranpreet Kaur
issued: 2024-07-22T16:22:56Z
issued: 2024
related degree: Master of Science - MSc
degree grantor: University of British Columbia
description: Chronic primary systemic vasculitis (PSV) describes a group of rare and potentially life-threatening disorders characterized by the inflammation and necrosis of blood vessels in one or more vital organs. In children, PSV typically affects small blood vessels, with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) being the most common form. Compared to adult-onset AAV, AAV in children is more likely to be severe at outset and present with multi-organ involvement. Furthermore, more than 80% of children exhibit kidney involvement at initial presentation, and approximately one-third develop permanent kidney damage within the first year. Due to the rarity of childhood-onset AAV, most knowledge of the disease is obtained by studying adult-onset AAV. In adults with AAV, certain autoantibodies and urine proteins have been identified as biomarkers of risk for severe disease, kidney involvement, and relapse, and are now used to guide treatment decisions. The intent of this thesis is to assess the utility of these adult-derived tools in childhood-onset vasculitis. Using the single largest repository of retrospective data and biosamples from children with vasculitis stored at the University of British Columbia and the BC Childrenâs Hospital Research Institute, this thesis reports, as primary findings, a higher likelihood of presenting with kidney involvement for children who are seropositive for ANCA directed towards myeloperoxidase (MPO) compared to those with ANCA towards proteinase-3 (PR3) and those without ANCA against either autoantigen, while neither ANCA seropositivity nor specificity were informative of the risk of relapse. Additionally, none of the three urine proteins (usCD163, usCD25, uMCP-1) identified as markers of kidney disease activity in adult-onset AAV exhibited a similar utility in the pediatric population. The findings from this thesis research identify potential differences in the utility of adult-derived biomarkers between childhood-…
aggregated CHO: https://circle.library.ubc.ca/rest/handle/2429/88664
note: EVALUATING THE UTILITY OF ADULT-DEFINED BIOMARKERS IN CHILDHOOD-ONSET CHRONIC PRIMARY SYSTEMIC VASCULITIS by Simranpreet Kaur Mann B.Sc., Integrated Sciences, University of British Columbia, 2022 A THESIS SUBMITTED IN PARTIAL FULFILLMENT OF THE REQUIREMENTS FOR THE DEGREE OF MASTER OF SCIENCE in THE FACULTY OF GRADUATE AND POSTDOCTORAL STUDIES (Microbiology and Immunology) THE UNIVERSITY OF BRITISH COLUMBIA (Vancouver) July 2024 © Simranpreet Kaur Mann, 2024 ii The following individuals certify that they have read, and recommend to the Faculty of Graduate and Postdoctoral Studies for acceptance, the thesis entitled: Evaluating the utility of adult-defined biomarkers in childhood-onset chronic primary systemic vasculitis submitted by Simranpreet Kaur Mann in partial fulfilment of the requirements for the degree of Master of Science in Microbiology and Immunology Examining Committee: Dr. Kelly Brown, Associate Professor, Dept. of Pediatrics, Division of Rheumatology, UBC Supervisor Dr. Donald Sin, Professor, Faculty of Medicine, Division of Respiratory Medicine, UBC Supervisory Committee Member Dr. Maria Tokuyama, Assistant Professor, Dept. of Microbiology and Immunology, UBC Additional Examiner Additional Supervisory Committee Members: Dr. Stuart Turvey, Professor, Faculty of Medicine, Dept. of Pediatrics, UBC Supervisory Committee Member iii Abstract Chronic primary systemic vasculitis (PSV) describes a group of rare and potentially life-threatening disorders characterized by the inflammation and necrosis of blood vessels in one or more vital organs. In children, PSV typically affects small blood vessels, with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) being the most common form. Compared to adult-onset AAV, AAV in children is more likely to be severe at outset and present with multi-organ involvement. Furthermore, more than 80% of children exhibit kidney involvement at initial presentation, and approximately one-third develop permanent…
has type: Thesis/Dissertation
date issued: 2024-11
is shown at: 10.14288/1.0444191
language: eng
degree discipline: Microbiology and Immunology
provider: Vancouver : University of British Columbia Library
publisher: University of British Columbia
rights: Attribution-NonCommercial-NoDerivatives 4.0 International
rights URI: http://creativecommons.org/licenses/by-nc-nd/4.0/
scholar level: Graduate
contributor: Brown, Kelly L.
type: Text
identifier URI: http://hdl.handle.net/2429/88664