Pediatric Vasculitis Initiative

Over a 3-year period, we anticipate enrollment and collection of clinical data from as many
as 600 children with various forms of childhood vasculitis, with approximately one third
(200) of those children also contributing biological samples for study.

For children with vasculitis who are enrolled in the study, clinical information will be
obtained from the medical chart from the time of diagnosis, post-induction (3-6 months post
diagnosis) visit, 12-month clinic visit, and their most recent clinic visit or last clinic
visit before discharge to adult care (ie. final outcome visit). Information that will be
collected includes laboratory test results, biopsy and imaging results, disease activity,
clinical history, and medications. Blood, urine, and saliva samples will also be collected at
each clinic visit. If the subject experiences a disease flare, clinical data and biological
samples will be collected at the time of the flare and at a later date when the disease
remits.

The PedVas study is linked to an adult vasculitis initiative called DCVAS: Diagnosis and
Classification Criteria in Vasculitis. Our DCVAS co-investigators and collaborators will
recruit up to 250 adults at or near the time of diagnosis of the following forms of
vasculitis: GPA, MPA, EGPA, TA, and UCV. Clinical data will be collected as part of the DCVAS
study; this includes information such as laboratory test results, disease activity, and
clinical history. Blood will also be collected and analyzed in parallel with samples
collected from children with vasculitis. Finally, a DNA-biobank will be created and will
house samples from approximately 700 adults and representing all forms of vasculitis.
Recruitment will proceed according to DCVAS approved protocols and it will be conducted at
participating DCVAS centres after the patient has formally consented to participation in the
DCVAS study.

Healthy volunteers from the community will be recruited to participate in this study by word
of mouth and recruitment posters. Participation for children involves a one-time donation of
blood and a urine sample, while adults may donate blood and urine up to 4 times over the
course of 18 months.

All biological samples will be processed and analyzed in Vancouver at the Child and Family
Research Institute and at the University of British Columbia. Detailed data will be collected
in electronic format and include demographic variables, socioeconomic status, detailed
clinical history & physical findings, anthropometric measures, and measures of disease
activity. All data for systemic vasculitis patients will be directly entered at each site
into a secure, online, web-based data entry system called REDCap which is managed through the
data management centre at the University of British Columbia in Vancouver. All CNS vasculitis
data will be entered into the Brainworks database which is managed by the data management
team at the Hospital for Sick Kids in Toronto.

Inclusion criteria for vasculitis subjects:

- Diagnosed with ANCA-associated vasculitis (AAV: such as Granulomatosis with
Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA) and
Microscopic Polyangiitis (MPA)), Primary Angiitis of the Central Nervous System
(PACNS), Unclassified vasculitis, Takayasu's Arteritis (TA) or Polyarteritis Nodosa
(PAN) before age 18

Inclusion criteria for healthy controls:

- Healthy adult or child

Exclusion Criteria for vasculitis subjects:

- Diagnosed with other vasculitis subtypes not listed above

- More than 20 years of age

Exclusion criteria for healthy controls:

- Donated greater than 20 ml of blood in the previous three weeks

- Has an immune disorder or blood borne infectious diseases (such as HIV or Hepatitis)

- Has vasculitis, multiple sclerosis, diabetes, an autoimmune disease, a thyroid
condition, or other chronic conditions involving the heart, lungs, gut or kidney

- Has a previous history of anaemia or abnormal blood clotting

- Has a current or previous drug abuse problem