Granulomatosis with polyangiitis (Wegener's granulomatosis)

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.

Symptoms of GPA

GPA can cause a range of symptoms depending on which parts of the body are affected.

It can cause:

• general symptoms such as tiredness, a high temperature (fever), weakness, loss of appetite, weight loss and joint pain
• ear, nose and throat problems such as a blocked or runny nose, nosebleeds, crusts around the nostrils, face pain (sinusitis), earache and hearing loss
• lung problems such as a cough that doesn't go away, shortness of breath, wheezing and chest pain
• kidney problems such as blood in pee, high blood pressure and inflammation of the kidneys (glomerulonephritis)
• skin problems such as rashes, lumps and small purple spots
• eye problems such as irritated eyes (conjunctivitis), swollen eyelids and double vision 
• gut problems such as tummy pain, diarrhoea and blood in poo

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.

When to get medical advice

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.

Tests for GPA

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:

• asking about your symptoms and examining the affected parts of your body
• a urine test to check how well your kidneys are working
• a blood test to look for substances called ANCAs (antineutrophil cytoplasmic antibodies), which are thought to be involved in GPA
• removing a small sample of tissue from an affected area (a biopsy) and checking it for signs of inflammation
• an X-ray, CT (computerised tomography) scan or MRI (magnetic resonance imaging) scan to look at affected parts of the body

Treatments for GPA

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.

1) Bringing the condition under control

Treatment first aims to bring the symptoms of GPA under control. This usually involves:

• having injections of a medicine called cyclophosphamide every two or three weeks, or taking it as tablets every day (occasionally, other medicines – such as methotrexate, mycophenolate mofetil or rituximab – may be used instead) 
• taking steroid tablets every day or having steroid injections into your blood at the same time as the cyclophosphamide injections

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.

2) Keeping the condition under control

Once your condition is under control, the treatment aims to stop your symptoms coming back. This usually involves:

• stopping treatment with cyclophosphamide
• taking tablets of a less powerful medicine that dampens the immune system, such as methotrexate or azathioprine
• taking steroid tablets every day

This stage of treatment typically lasts between two and five years.

3) Treating symptoms if they come back

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.

Living with GPA

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.

Support and advice if you have GPA

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.

Causes of GPA

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.

Information about you

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.

Find out more about the register.