GPA can cause a range of symptoms depending on which parts of the body are affected.
General symptoms include:
Ear, nose and throat problems:
If it's not treated, GPA can cause permanent damage to some parts of the body. For example, it can change the shape of the nose or stop the kidneys working properly.
See a GP if you have symptoms of GPA, especially if they do not go away.
The GP can do some simple checks to try to find out what's causing your symptoms and can refer you to a hospital specialist for further tests, if needed.
If you've already been diagnosed with GPA, contact your doctor if any of your old symptoms come back or you get any new symptoms. Your treatment may need to be changed.
GPA can be difficult to diagnose. It causes a range of symptoms that are often similar to other common conditions.
A specialist doctor may:
GPA is thought to be caused by a problem with the immune system. It's treated with medicines that reduce the activity of the immune system.
There are 3 main stages of treatment.
The first aim of treatment is to bring the symptoms of GPA under control. This usually involves:
These are all strong medicines, so make sure you discuss the possible side effects with your doctor.
Some people also need a plasma exchange, where a machine is used to filter the blood to remove the harmful antibodies linked to GPA.
This first stage of treatment lasts until your symptoms are under control, which usually takes a few months.
Once the condition is under control, treatment aims to stop your symptoms coming back. This usually involves:
This stage of treatment typically lasts for 2 to 5 years.
If your symptoms come back or you get new symptoms (a relapse) at any point, your treatment may be changed or restarted.
For example, your dose of steroids may be increased, and you may need to have more cyclophosphamide injections, start treatment with rituximab, or possibly have a course of plasma exchange.
GPA is a serious condition but, with treatment, it can usually be kept under control.
You might need to take medicine for several years and you'll have regular check-ups in case your symptoms come back.
About half of people with GPA have a relapse within a few years of their treatment stopping. Further treatment can help bring the condition back under control if this happens.
If GPA is severe or is not treated quickly, there is a risk that life-threatening problems could develop, such as permanent damage to the kidneys that may require a kidney transplant.
This is why it's important to contact your doctor as soon as possible if your symptoms come back or you start getting new ones.
For more information and advice, you may find it useful to check the Vasculitis UK website.
Vasculitis UK is an organisation for people with vasculitis (inflammation of the blood vessels). GPA is a type of vasculitis.
The Vasculitis UK website has information about living and coping with vasculitis, including advice about general health, benefits and insurance.
The exact cause of GPA is unknown.
It may be due to problems with the immune system, which makes it attack and inflame the blood vessels. But it's not clear why this happens.
People with GPA may have a gene that makes them more likely to get the condition. It might then be triggered by something like a virus or bacterial infection, although this has not been proven.
Genes alone are not responsible for GPA because it's very unusual for 2 people in the same family to have it.
If you have GPA, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS).
The NCARDRS helps scientists look for better ways to prevent and treat GPA. You can opt out of the register at any time.