Primary Childhood Vasculitis

Chronic primary vasculitis describes a group of rare, life-threatening diseases that have in common inflammation of blood vessels in vital organs such as kidneys, lungs and brain.

The challenge in treating children with vasculitis is balancing the risk of under treating aggressive disease with the potential damaging side-effects of prolonged use of drugs.

This project uses an established international web-based registry to collect clinical information from 50 or more centers worldwide, with approximately 20 centres contributing paired biological samples (DNA, RNA, plasma, serum, urine). The goal is to elucidate subclinical (molecular/cellular) features which help to classify, define and direct optimal treatment for children with vasculitis.

Subtypes of primary vasculitis classified by affected vessel size (Source:
Pathogenic consequences of vasculitis on vascular structure (Source:

The Pediatric Vasculitis Initiative (PedVas)


The PedVas study is an international study, headed by Dr. David Cabral, based at BC Children’s Hospital and includes collaborators in Canada, US, and various countries across the globe. The project is funded by the Canadian Institutes of Health Research (CIHR). This study is examining childhood chronic vasculitis, a set of inflammatory diseases affecting blood vessels in body organs such as the kidneys, lungs and brain.

Selected Publications

  • Different disease endotypes in phenotypically similar vasculitides affecting small-to-medium sized blood vessels

    Gill EE, Smith ML, Gibson KM, Morishita KA, Lee AHY, Falsafi R, Graham J, Foell D, Benseler S, Ross C, Luqmani R, Cabral DA, Hancock REW, and Brown KL on behalf of the PedVas Initiative Investigators.

    (2021) Frontiers in Immunology 12: 638571. PubMed

  • Autoantibodies against lysosome associated membrane protein-2 (LAMP-2) in pediatric chronic systemic vasculitis

    Gibson KM, Kain R, Luqmani R, Ross CJ, Cabral DA, and Brown KL on behalf of the PedVas Initiative Investigators.

    (2021) Frontiers in Immunology 11: 624758. PubMed

  • Adenosine Deaminase 2 activity negatively correlates with age during childhood

    Bowers SM, Gibson KM, Cabral DA, and Brown KL.

    (2020) Pediatric Rheumatology 18(1): 54. PubMed

  • Anti-neutrophil cytoplasmic antibodies (ANCA): Antigen interactions and downstream effects

    Sundqvist M, Gibson KM, Bowers SM, Niemietz I, and Brown KL.

    (2020) Journal of Leukocyte Biology 108(2): 617-626. PubMed

  • Identification of novel ADA2/CECR1 gene variants and varied clinical phenotype in pediatric vasculitis

    Gibson KM, Morishita K, Dancey P, Moorehead P, Drogmoller B, Shan X, Graham J, Hancock REW, Foell D, Benseler S, Luqmani R, Yeung R, Shenoi S, Bohm M, Rosenberg A, Ross CR, Cabral DA, Brown KL on behalf of the PedVas study group.

    (2019) Arthritis & Rheumatology In Press. Apr 22. PubMed

  • Monocyte-derived interleukin-1 as the driver of S100A12-induced sterile inflammatory activation of human coronary artery endothelial cells: Implications for the pathogenesis of Kawasaki Disease

    Armaroli G, Verweyen E, Pretzer C, Kessel K, Hirono K, Ichida F, Okabe M, Cabral DA, Foell D, Brown KL and Kessel C.

    (2019) Arthritis & Rheumatology 71(5): 792-804. PubMed

  • S100A12 and common inflammatory markers improve assessment of disease activity in a PedVas cohort of childhood systemic vasculitis

    Brown KL, Lubieniecki JM, Armaroli G, Kessel K, Gibson KM, Graham J, Liu D, Hancock REW, Ross CJ, Benseler S, Luqmani R, Cabral DA, Foell D and Kessel C on behalf of the PedVas study group.

    (2018) Frontiers in Pediatrics 6: 341. PubMed

  • Clinical practice variation and need for pediatric-specific treatment guidelines among rheumatologists caring for children with ANCA-associated vasculitis: an international clinician survey

    Westwell-Roper, C, Lubieniecka JM, Brown KL, Morishita KA, Mammen C, Wagner-Weiner L, Yen E, Li, SC, O’Neil K, Lapidus SK, Brogan P, Cimaz R, Cabral DA for the ARChiVe Investigators Network within the PedVas initiative.

    (2017) Pediatric Rheumatology Online Journal 15(1): 61. PubMed