Grant: Project Grant

Vasculitis is a rare disease where the body's immune system attacks blood vessels, leading to damage in important organs like the kidneys. By the time most children are diagnosed, they already have poor kidney function, and 1 in 4 need dialysis right away. Even with strong medications, half of children will have long lasting kidney damage and as many as 30% will need a kidney transplant. There is an urgent need to identify, at diagnosis, those children at highest risk for the worst outcomes so treatments can be tailored to their needs. Researchers have recently developed tools to predict how vasculitis in adults will affect the kidneys, but these tools haven't been tested in children. Vasculitis in children has some important differences and children may have a stronger ability to recover, thus, the adult- based tools may not accurately predict their risk. Also, the tools were designed to predict kidney failure five to ten years after diagnosis. This is a problem since, in children, the fate of the kidneys is usually determined in the first 12 months after diagnosis and, aside from total kidney failure, permanently poor kidney function (called chronic kidney disease) can seriously impact a child's health and future quality of life. Over the past 10+ years, our research team has collected detailed medical information and samples from the largest group of children with vasculitis in the world. Our study will (1) test and tailor the existing 'adult' risk tools using data from >100 children with the disease, and (2) use urine to discover early warning signs of poor functional recovery of the kidneys. Ultimately, this work could tell us which children have a high versus low likelihood of bad kidney function (failure or chronic disease) one year after diagnosis, thus transforming how we care for children with this serious disease.