GCA Rare Disease Bioresource and GCA phenotyping study Autoimmune Vasculitis Rare Disease Bioresource and Vasculitis phenotyping study

Summary

There are many different types of vasculitis which are categorised based on the size of the blood vessel involved. For example, small blood vessels are involved in anti-neutrophil cytoplasm antibody-associated vasculitis, while Takayasu arteritis and giant cell arteritis affect large blood vessels, such as the aorta. Autoimmune vasculitides are rare diseases, with overlapping clinical features. Our understanding of the mechanisms leading to activation and switching off different components of the immune system, antibodies and platelets in vasculitis has increased over the past two decades. A key clinical challenge when caring for people with vasculitis is balancing control of the immune system (prevents complications) against over-suppression (leading to infections). Although many new treatments are becoming available, these tend to undergo clinical trials in only one form of vasculitis, whereas patients with all forms of vasculitis may benefit from these new medications. In this study we wish to understand the biological processes that are happening in all types of vasculitis and how that relates to the type of vasculitis, activity of vasculitis and treatment. This will allow us to see if the same immune, vascular and genetic signatures are seen in different types of vasculitis. To determine vasculitis specific biological processes, we will use related conditions such as Adult onset stills disease and autoinflammatory syndromes as disease comparators. With greater understanding of the underlying biology we hope to build a case for increased access to treatments across a wider range of vasculitides and identify new treatment opportunities. We will also investigate whether some of the tests that are being performed will be helpful in the clinic to try and determine who might respond to specific treatments, or even who is in complete disease remission where it might be possible to withdraw treatment.