Creating a better understanding of rare auto-immune diseases

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John Mills

Founder & Director of Operations, Vasculitis UK

For people living with rare autoimmune diseases such as ANCA-associated vasculitis, patient associations are providing invaluable support

Vasculitis is a collective term for a group of 18 rare auto-immune diseases, all of which involve inflammation of the lining of the blood vessels.

As every part of the body has a blood supply, vasculitis has the potential to affect every organ and every bodily system and function.

Each type of vasculitis is rare, but collectively, vasculitis is classified as “uncommon”. Some types of vasculitis involving the kidneys and/or lungs, such as ANCA-associated vasculitis (AAV), can be rapidly fatal if not diagnosed promptly and treated appropriately.

There is no cure for vasculitis, but with prompt and appropriate care the disease can be controlled.

Different types of vasculitis affect mainly specific age groups. Thus, Kawasaki disease affects infants and very young people; where as giant cell arteritis (GCA) affects predominantly those aged 70 and over. Whilst AAV appears to affect the older generation more it can also present in younger patients.

Finding treatment options

There is no cure for vasculitis, but with prompt and appropriate care the disease can be controlled.

The general approach of treatment is to suppress the over-active immune response using a combination of steroids and immune-suppressing drugs. The recent introduction of “targeted” monoclonal antibodies (biologics) has transformed treatment outcomes in many instances.

The cause of vasculitis remains obscure but is generally considered to be due to an individual’s genetic predisposition combined with some sort of environmental trigger, such as an infection or exposure to a chemical or drug. Recent advances in genomics might support this theory.

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