Evaluating renal and pulmonary disease in pediatric-onset ANCA-associated vasculitis : disease course, outcomes, and predictors of outcomes
department or school: Medicine, Faculty of
department or school: Obstetrics and Gynaecology, Department of
data provider: DSpace
degree campus: UBCV
creator: Toor, Kirandeep
issued: 2025-01-09T17:57:17Z
issued: 2025
related degree: Master of Science - MSc
degree grantor: University of British Columbia
description: Pediatric-onset anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare, systemic disease involving inflammation and damage to small and medium sized blood vessels, primarily affecting the lungs and kidneys. The rarity of AAV in childhood precludes pediatric-specific clinical trials, thus treatment strategies have been adopted from trials in adult-onset AAV. Although these ‘adult’ treatment approaches have substantially decreased mortality in children with AAV, significant concerns persist around the toxicity risks specific to children and the appropriateness of applying adult-based recommendations to pediatric-onset disease. Our previous research demonstrated that, compared to adult-onset AAV, pediatric-onset AAV is more likely to be severe and have major organ dysfunction, such as significantly reduced renal function and severe lung disease. The intent of this thesis is to investigate the course of renal and pulmonary disease in children with AAV in the first 12-months, and to evaluate any predictors of these outcomes. This thesis research used both retrospectively and prospectively collected clinical data from the largest multi-center, international registry of children with vasculitis hosted at the University of British Columbia and the BC Children’s Hospital Research Institute. This thesis reports that despite the majority of patients receiving aggressive treatment, two-thirds of patients have reduced kidney function at 12 - and 24–month follow-up and more than one-third of patients have evidence of permanent kidney damage at 12-months. Kidney function at diagnosis (eGFR) is a strong predictor of kidney function at 12-months. Additionally, >50% of children with AAV present with pulmonary disease; however, the majority achieve inactive disease by 12-months, and few are left with pulmonary damage. This research characterized pulmonary and renal disease course, described early outcomes, and demonstrated the utility of using eGFR as a biom…
aggregated CHO: https://circle.library.ubc.ca/rest/handle/2429/90074
note: EVALUATING RENAL AND PULMONARY DISEASE IN PEDIATRIC-ONSET ANCA-ASSOCIATED VASCULITIS: DISEASE COURSE, OUTCOMES, AND PREDICTORS OF OUTCOME by Kirandeep Toor Honours B.Sc., Life Sciences, McMaster University, 2021 A THESIS SUBMITTED IN PARTIAL FULFILLMENT OF THE REQUIREMENTS FOR THE DEGREE OF MASTER OF SCIENCE in THE FACULTY OF GRADUATE AND POSTDOCTORAL STUDIES (Women+ and Children’s Health Sciences) THE UNIVERSITY OF BRITISH COLUMBIA (Vancouver) December 2024 © Kirandeep Toor, 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 renal and pulmonary disease in pediatric-onset ANCA-associated vasculitis: disease course, outcomes, and predictors of outcome submitted by Kirandeep Toor in partial fulfilment of the requirements for the degree of Master of Science in Women+ and Children’s Health Sciences Examining Committee: Dr. Kimberly Morishita, Clinical Associate Professor, Dept. of Pediatrics, Division of Rheumatology, UBC Co-supervisor Dr. Kelly Brown, Associate Professor, Dept. of Pediatrics, Division of Rheumatology, UBC Co-supervisor Dr. Diane Lacaille, Professor, Dept. of Medicine, Division of Rheumatology, UBC Supervisory Committee Member Dr. Jonathan Rayment, Clinical Assistant Professor, Faculty of Medicine, Dept. of Pediatrics, UBC Additional Examiner iii Abstract Pediatric-onset anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare, systemic disease involving inflammation and damage to small and medium sized blood vessels, primarily affecting the lungs and kidneys. The rarity of AAV in childhood precludes pediatric-specific clinical trials, thus treatment strategies have been adopted from trials in adult-onset AAV. Although these ‘adult’ treatment approaches have substantially decreased mortality in children with AAV, significant concerns persist around the toxicity risks specific to children and the appropria…
has type: Thesis/Dissertation
date issued: 2025-05
is shown at: 10.14288/1.0447707
language: eng
degree discipline: Women+ and Children’s Health Sciences
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: Morishita, Kimberly
contributor: Brown, Kelly L.
type: Text
identifier URI: http://hdl.handle.net/2429/90074