Grant: Operating Grant

Severe, anti-neutrophil cytoplasm antibody associated vasculitis (AAV) is a serious disease of the immune system that affects approximately 34,000 Canadians. AAV is important because up to 50% of patients will die or develop kidney failure within 5 years of diagnosis. The main treatments for AAV suppress the immune system to control the disease. However, this results in up to 50% suffering a severe infection and the need to balance enough immune suppression to control the AAV but not so much as to cause an infection. Canadian researchers lead the largest research study in the world in AAV which will determine the best way to use plasma exchange, a way of removing part of the immune system temporarily, and anti-inflammatory steroids (prednisone) to treat AAV. This study is called PEXIVAS and it will finish recruiting patients in 2016. At the end of PEXIVAS we will understand the better understand the best initial treatment of AAV. However, we will not know much about the long term effects of plasma exchange and prednisone on AAV and patient health. We will also not have much information on how subsequent treatments for AAV after plasma exchange and prednisone, affect the outcomes for these patients. We will continue to follow PEXIVAS patients for between 5 and 10 years after their first treatment. This will help determine the effect of plasma exchange and prednisone on recurrences of AAV, kidney function, heart health, cancer and death in the long-term. It will also help determine the effect of the treatments given after plasma exchange and prednisone on these health outcomes.