Granulomatosis with polyangiitis (Wegener's granulomatosis) 

  • Overview

Introduction 

Chest X-ray of a woman with Wegener's granulomatosis, showing damage to the lungs 

Granulomatosis with polyangiitis (GPA) is an uncommon disorder in which the walls of blood vessels become inflamed (swollen) and leaky, leading to poor blood flow to tissues throughout the body.

It is a serious condition that can be fatal if left untreated.

GPA can cause a wide range of symptoms, including sinusitis, persistent fever and night sweats.

The inflammation of the blood vessels (known as 'vasculitis') caused by GPA mainly affects the nose, sinuses, ears, lungs and kidneys, although other organs can be affected too, including the brain, skin, nerves, heart and bowels.

Eventually, GPA can lead to tissue damage and organ failure.

If you have been diagnosed with GPA and suddenly develop any of the below symptoms, see your GP immediately – you are probably experiencing a relapse (return of the disease).

Note that GPA used to be called 'Wegener's granulomatosis' and was only renamed recently.

What are the symptoms?

Typical symptoms of GPA are:

Some patients may be at risk of blood clots in the leg (deep vein thrombosis) or in the lungs (pulmonary embolism).

Who is affected?

Around 500 men and women are diagnosed with GPA each year in the UK. Symptoms can start at any age, including in childhood, although the condition is usually triggered in middle age (in the 40s and 50s).

What is the cause?

The exact cause of GPA is unknown. It is thought that GPA is an autoimmune disease, which means the body's immune system mistakenly attacks its own tissues.

As with other autoimmune disorders, it's probable that people with GPA have a gene that makes them more likely to develop the condition. GPA is then probably triggered by an additional factor, such as an infection with bacteria or a virus.

Genes alone are not responsible for the condition. It's very unusual for GPA to occur in two people in the same family.

How is it diagnosed?

Your doctor may suspect GPA if you feel unwell, have a persistent fever and have lost weight, and one or more of the following regions of your body is affected:

  • your nose, ears and sinuses – for example, you have persistent earache, nosebleeds or sinusitis 
  • lungs – for example, you have difficulty breathing or are coughing up blood, or your chest X-ray shows lung abnormalities 
  • kidneys – for example, there is blood in your urine or protein in your urine

Blood tests may be done to test levels of ANCAs (antineutrophil cytoplasmic antibodies). These are antibodies that the immune system of people with GPA uses to attack the body's own blood vessels. However, this test can sometimes come back negative in people with GPA, so cannot be relied upon on its own to diagnose the condition.

A tissue sample of the affected area may also be taken and looked at under the microscope for evidence of inflammation. In GPA, the white blood cells clump together to form tiny lumps called granulomas.

Urine tests may be done to look for blood or protein in your urine, a sign that GPA may have affected your kidneys.

Other helpful tests may include:

How is it treated?

You will need high doses of drugs to dampen down your immune system and bring the inflammation under control.

Typically, you will be given steroid medications (prednisone) plus cyclophosphamide, a cancer drug that weakens your immune system.

Cyclophosphamide is normally given intravenously (directly into a vein) every two to four weeks. Steroid medication is given alongside this, but the dose of steroids is gradually reduced over time and stopped after three to nine months.

If the condition has started to respond to treatment and is getting a bit better, your doctor may switch you to a milder immune-suppressing drug such as methotrexate or azathioprine.

Cyclophosphamide and steroids are powerful drugs, so discuss the side effects with your doctor. Read more about the side effects of steroid medication.

People with GPA may also need antibiotics to take over a long period, to help prevent infections.

A small group of people with GPA may not respond to the above treatment and may need stronger medications to control the disease. Medical researchers are always searching for better and safer treatment options for GPA.

Surgery

Once the disease is under control with medication, some people may need surgical treatment for:

  • nose deformity
  • narrowing of the airways
  • obstruction of the tear ducts
  • ear problems that require the insertion of grommets
  • kidney failure where a kidney transplant is needed

Outlook

The earlier the disease is diagnosed and treated, the better the outlook.

Once the disease gets better and treatment is stopped, about half of people with GPA will have a relapse (the disease returns). This usually happens within two years of stopping treatment.

Most people with GPA lead normal lives. If you have GPA you will need to take your medicines regularly, have blood tests every few months and continue to see your doctor regularly for signs of a relapse.

Complications, if they occur, are usually the result of a delay in diagnosis and a lack of treatment. If left untreated, people with GPA may develop inflammation in the respiratory tract and kidneys, which can eventually lead to lung damage and kidney failure. Other possible complications include sore eyes and a hole inside the nose. Speak to your doctor if you are concerned about complications.

Page last reviewed: 03/09/2012

Next review due: 03/09/2014

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Comments

The 2 comments posted are personal views. Any information they give has not been checked and may not be accurate.

widget77 said on 21 February 2014

A diagnosis of Wegner's can be terrifying, especially reading a lot of the information on the web. My husband was diagnosed aged 27, he went from super fit to coughing up blood, he was ventilated and we were told to say goodbye, they managed to get him onto ECMO then his kidneys failed, lungs collapsed and he had 4 cardiac arrests. The team at Papworth and Addenbrookes were amazing once they diagnosed him with WG he was given plasmapherisis, IVig, Prednisone and Rituximab. After 2 months in hospital his lungs, kidneys and heart all came back. He was discharged in the June, stayed on prednisone until the end of that year and continued on Rituximab infusions once every 6mths for 2years. He is now in remission with no lasting damage, he's super fit and healthy. Rituximab meant he was never immunosuppressed so he had no infections after leaving hospital. I just wanted to share with anyone who may be visiting this page with a diagnosis to let you know it's not a death sentence, it can be treated and you can have a normal life.

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Victoria Wollfe said on 13 December 2013

I have lived with a partner with late diagnosis Wegeners for 3 years

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