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Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a rare condition where 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 adults and older people.

GPA can be very serious but, with medicine, most people are able to manage the symptoms and keep the condition under control.


General symptoms include:

Ear, nose and throat problems:

Lung problems:

Kidney problems:

Skin problems:

  • rashes
  • lumps
  • small purple spots

Eye problems

Gut problems:


See a GP if you have symptoms of GPA, especially if they do not go away.


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:

  • ask about your symptoms and examine the affected parts of your body
  • do a urine test to check how well your kidneys are working
  • remove a small sample of tissue (a biopsy) and check it for signs of inflammation
  • organise an X-ray, CT scan or MRI scan to look at the affected parts of the body in more detail


There are 3 main stages of treatment.

Stage 1: Bringing the condition under control

The first aim of treatment is to bring the symptoms of GPA under control. This usually involves:

  • having injections of a medicine called cyclophosphamide every 2 or 3 weeks, or taking it as tablets every day (occasionally, other medicines – such as methotrexate, mycophenolate mofetil or rituximab – may be used instead)

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.


Stage 2: Keeping the condition under control

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

  • stopping treatment with cyclophosphamide
  • taking medicine that helps "calm" (or control) your body's immune system, such as an immunosuppressant like methotrexate or azathioprine
  • taking steroid tablets every day

This stage of treatment typically lasts for 2 to 5 years.

Stage 3: Treating symptoms if they come back


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.


You might need to take medicine for several years and you'll have regular check-ups in case your symptoms come back.


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.




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.

National Congenital Anomaly and Rare Disease Registration Service


The NCARDRS helps scientists look for better ways to prevent and treat GPA. You can opt out of the register at any time.

GOV.UK has more information about the register