Granulomatosis with polyangiitis (Wegener's granulomatosis) 

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

Ratings

How helpful is this page?

Average rating

Based on 74 ratings

All ratings

Add your rating

Comments

The 6 comments posted are personal views. Any information they give has not been checked and may not be accurate.

ShannonPayne said on 17 September 2014

Hi David88, thank you so much for giving me a little bit of hope, each day I wake up grieving the loss of my healthy child, she struggles daily with the effects of the prednisolene and is currently feeling very low. We spent a day in hospital attempting a rituximab infusion but it had to be abandoned due to severe liver pain, however they decided to try again a few days later with a half-bag and despite pain she managed to tolerate it, we have the 3rd and 4th half-bags scheduled over the next 2 weeks. They have reduced the prednisolene from 60mg to 40mg and hope to reduce it further after the 4th half-bag. I am glad you mentioned keeping hydrated, it was mentioned by the doctors but as there are so many things to remember at this stage I hadn't really hung on to this advice, I will do now though! I worry daily about the long term effects of this illness, my daughter is a clever girl, an A* student, but the steroids have altered her attitude to studying, plus all her friends keep telling her that she will have a rubbish time at university as she won't be able to drink at parties etc. In the grand scheme of things this isn't important, but at the moment from her perspective it all seems doom and gloom. I will print off your response and share it with her tonight, thank you so much for taking the time to respond, I would have responded earlier but we have been in and out of hospital.

Report this content as offensive or unsuitable

David88 said on 19 July 2014

Hi Shannon. Can imagine you're terrified, my mum was the same when I was diagnosed with WG when I was 15 (i'm now 26). You should try not to be overwhelmed with worry though, there are numerous treatments for WG; in my experience some are better than others, but I and many others who have suffered with this disorder now live normal healthy lives.
I presented with ear, eye, mouth pain and issues. Severe joint pain. I was coughing up blood, had a very swollen spleen and kidney problems.
I too had a kidney biopsy done. My results showed 50% damage. Although the kidneys don't heal completely after treatment, they almost do. I now only have a trace amount of protein in my urine and no blood and suffer no issues with it. You've probably been told this, but your daughter will need to try keep well hydrated at all times from now on to ease the strain on her kidneys.
My first treatment was Cyclophosphamide, steroids and various other supplements. I was put on a high dose of steroids, unnecessarily high I was later told. I was on 75mg daily. When I relapsed a year later I was only put on 40mg daily and it offered the same benefit with less severe side affects. Prednisone offers great relief, quickly. However it also has significant and unfortunate side affects and my advice would be not to be scared to question the dose if you think it may be too high.
I relapsed about 9 months later and was then switched to MMF, like your daughter. This worked fairly quickly and I went into remission and managed to lead a fairly normal teenage life, with my medication gradually reduced over the years.
I relapsed again when I was 19 and was given a drip of rituximab once a week for 4 weeks, with no other medication and have been in full remission since. I have led a normal, healthy life, medication free for 7 years. I would recommend you ask your daughters doctor about rituximab treatment. It has been a miracle cure for me.

If you want to know more, just ask. All the best

Report this content as offensive or unsuitable

David88 said on 19 July 2014

Hi Shannon. Can imagine you're terrified, my mum was the same when I was diagnosed with WG when I was 15 (i'm now 26). You should try not to be overwhelmed with worry though, there are numerous treatments for WG; in my experience some are better than others, but I and many others who have suffered with this disorder now live normal healthy lives.
I presented with ear, eye, mouth pain and issues. Severe joint pain. I was coughing up blood, had a very swollen spleen and kidney problems.
I too had a kidney biopsy done. My results showed 50% damage. Although the kidneys don't heal completely after treatment, they almost do. I now only have a trace amount of protein in my urine and no blood and suffer no issues with it. You've probably been told this, but your daughter will need to try keep well hydrated at all times from now on to ease the strain on her kidneys.
My first treatment was Cyclophosphamide, steroids and various other supplements. I was put on a high dose of steroids, unnecessarily high I was later told. I was on 75mg daily. When I relapsed a year later I was only put on 40mg daily and it offered the same benefit with less severe side affects. Prednisone offers great relief, quickly. However it also has significant and unfortunate side affects and my advice would be not to be scared to question the dose if you think it may be too high.
I relapsed about 9 months later and was then switched to MMF, like your daughter. This worked fairly quickly and I went into remission and managed to lead a fairly normal teenage life, with my medication gradually reduced over the years.
I relapsed again when I was 19 and was given a drip of rituximab once a week for 4 weeks, with no other medication and have been in full remission since. I have led a normal, healthy life, medication free for 7 years. I would recommend you ask your daughters doctor about rituximab treatment. It has been a miracle cure for me.

If you want to know more, just ask. All the best

Report this content as offensive or unsuitable

ShannonPayne said on 15 July 2014

My 14 year old has just been diagnosed with Granulomatosis with polyangiitis, after reading various articles on the internet (a bad idea I know) I am scared silly, can anybody give me a bit of reassurance based on personal experience? According to a preliminary kidney biopsy it appears about 1/3rd of her kidneys have been damaged, further tests will give more detail. She is taking 17 tablets a day consisting of 12 steroid, 2 for indegestion as a result of the steroid and 3 MMF.

Report this content as offensive or unsuitable

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.

Report this content as offensive or unsuitable

Victoria Wollfe said on 13 December 2013

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

Report this content as offensive or unsuitable

Long-term conditions

Living with a long-term condition, including healthcare, medicines and support

Pyoderma gangrenosum

A rare skin reaction to a disease or illness, which results in painful skin ulcers. Read more...