Granulomatosis with polyangiitis (GPA) is a rare condition in which the blood vessels become inflamed. It mainly affects the ears, nose, sinuses, kidneys and lungs.
Anyone can get it, including children, but it's most common in middle-aged or older people.
GPA can be very serious but, with medication, most people can keep it under control and live largely normal lives.
GPA used to be called Wegener's granulomatosis.
GPA can cause a range of symptoms depending on which parts of the body are affected.
It can cause:
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 your GP if you think you may have symptoms of GPA, especially if they don't go away.
Your 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 your get any new symptoms. Your treatment may need to be adjusted.
GPA can be hard to diagnose. It causes a range of symptoms and they are often similar to those of more common conditions.
A specialist doctor may need to do several checks and tests before they can diagnose GPA.
This may involve:
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.
Treatment involves three main stages.
Treatment first aims to bring the symptoms of GPA under control. This usually involves:
These are all powerful medicines, so make sure you discuss the possible side effects with your doctor.
Some people also need to have 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 your condition is under control, the treatment aims to stop your symptoms coming back. This usually involves:
This stage of treatment typically lasts between two and five 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.
Most people can lead largely normal lives, although you might need to take medication for several years and you'll have regular check-ups to monitor your condition 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 isn't 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 with vasculitis, including advice about things such as diet, benefits and insurance.
The exact cause of GPA is unknown.
It's thought to be due to something going wrong with the immune system, which makes it attack and inflame the blood vessels. But it's not clear why this happens.
It's likely that people with GPA 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 hasn't been proven.
Genes alone aren't responsible for GPA, as it's very unusual for it to occur in two people in the same family.
If you have GPA, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).
This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.
Page last reviewed: Sun Aug 2020 Next review due: Sun Aug 2020