Granulomatosis with polyangiitis (Wegener's granulomatosis) 

Introduction 

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

Coughing up blood

Read about causes of blood in your phlegm, and what you should do

Granulomatosis with polyangiitis (GPA) is a rare disease which causes the walls of the blood vessels to become inflamed (swollen).

It's a serious condition that can eventually lead to tissue damage and organ failure and can be fatal if left untreated.

However, there are effective treatments which mean most people can live relatively normal lives with the condition.

Until recently, GPA used to be called Wegener's granulomatosis.

Signs and symptoms

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.

This means the condition can have a wide range of symptoms, such as:

Some people with GPA may also be at risk of developing blood clots in their legs (deep vein thrombosis) or lungs (pulmonary embolism).

When to seek medical advice

If you have any of the above symptoms that are persistent or troublesome see your GP. They can arrange appropriate tests if they suspect GPA is the cause and, if appropriate, arrange referral to a specialist centre for treatment.

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

What causes GPA?

The exact cause of GPA is unknown. It is thought that GPA is likely to be 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, as it's very unusual for GPA to occur in two people in the same family.

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 usually first appears in middle-aged or elderly people.

How GPA is 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 areas 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 or protein in your urine

Blood tests may be done to test levels of ANCAs (antineutrophil cytoplasmic antibodies). These are antibodies thought to be involved in the process that leads to the blood vessels becoming inflamed.

However, this test can sometimes come back negative in people with GPA and positive in people who don’t have the condition, so it can't be relied upon on its own to diagnose the condition.

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

Computerised tomography (CT) scans and magnetic resonance imaging (MRI) scans may also be carried out to assess the condition of any organs or areas of the body that may be affected.

How GPA is treated

The aim of treatment for GPA is firstly to bring the condition under control (known as inducing remission), and then try to prevent the condition relapsing (known as maintaining remission).

For most people, this can be achieved using a number of different medications, which may need to be taken for a number of years. Medication administration is supervised through specialist centres in close liaison with your GP and is tailored to each individual patient.

Inducing remission

To begin with, you will usually need high doses of medication to dampen down your immune system and bring the inflammation under control.

Typically, you will be given a steroid medication called prednisolone to reduce the inflammation in addition to a medication called cyclophosphamide, which weakens your immune system.

Steroid medication is either given intravenously alongside this, or is taken by mouth (orally) on a daily basis.

Cyclophosphamide is normally given intravenously (directly into a vein) every two to three weeks.

In milder cases, an alternative oral immune-suppressing medication called methotrexate may be given instead of cyclophosphamide.

This treatment will continue – with your steroid dose gradually reduced over time – until you achieve remission, which in most people takes around three to six months.

Cyclophosphamide and steroids are powerful medications, so make sure you discuss the possible side effects with your doctor. Read more about the side effects of steroid medication.

Maintaining remission

Once remission is achieved, your treatment with cyclophosphamide will be stopped and you will be given a milder immune-suppressing medication such as methotrexate or azathioprine.

Your steroid medication may also be stopped, or you may continue taking it at a low dose.

This stage of treatment will usually last between two and five years and will also involve frequent check-ups to look for signs of a relapse.

Other treatments

Although the medications described above are effective for most people, some people with GPA will need alternative or additional treatment.

For example, you may be given long-term treatment with antibiotics alongside your other medications to prevent potentially serious infections, as the medications used to treat GPA can increase your chances of picking up an infection.

If you have severe GPA, cyclophosphamide is not suitable for you, or your condition relapses despite therapy, you may be offered a medication called rituximab instead. This medication works by destroying certain cells produced by the immune system that are thought to play a role in the inflammation of the blood vessels.

In particularly severe cases affecting the kidneys, a procedure called plasma exchange (plasmapheresis) may be recommended. 

During this procedure, you will be connected to a machine that removes some of your blood. A blood component called plasma will then be separated from the blood cells and removed, before the remaining blood cells are returned to your body. This process can remove some of the antibodies thought to contribute to the inflammation in GPA.

Once the disease is under control, some people may also need surgical treatment. For example, surgery may be needed to correct any nose deformities, narrowing of the airways or obstruction of the tear ducts that can occur as a result of the tissue damage caused by GPA.

If damage to the kidneys means they are no longer able to work properly, a kidney transplant may be needed.

Outlook

Although GPA can be life-threatening, early diagnosis and modern treatments can significantly improve the outlook.

Most people are able to achieve remission with medical treatment, although about half of people with GPA will have a relapse at some point. This usually happens within a few years of stopping treatment.

If you do have a relapse, you will usually need to start taking medication again or increase the doses of any medication you are already taking.

Most people with GPA are able to lead normal lives, although – in addition to taking medication regularly – you will need to have regular check-ups to monitor your progress and check for signs of a relapse.

Complications, if they occur, are often the result of a delay in diagnosis and a lack of treatment. If left untreated, people with GPA may develop severe inflammation in the respiratory tract and kidneys, which can eventually lead to life-threatening lung and kidney failure.




Page last reviewed: 09/09/2014

Next review due: 09/09/2016

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Comments

The 8 comments posted are personal views. Any information they give has not been checked and may not be accurate.

ShannonPayne said on 23 September 2014

Hi Michael, thanks for spending the time to tell me about your experience of this illness, I feel so helpless as all I want to do is take it away from my daughter and make her better, it's reassuring that kidney function can recover and that there are people out there willing communicate that things will improve. The hospital didn't mention that joint pain may be an issue, it certainly was crippling prior to diagnosis, so thanks for that information, I really appreciate it! If only we could turn the clock back and appreciate what we had, instead we have to learn to live with the illness and hopefully be able to say that my daughter lives a full and active life like you.

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Michael857 said on 19 September 2014

I'd also like to reassure Shannon. I was diagnosed in 2009, after a winter of severe joint pains, red eyes, and going almost deaf. I had grommets put in my ears which helped hugely. They found out I had only one kidney (a surprise!) which was being damaged. Kidney function has now fully recovered and I lead a full and active life now. I was treated with cyclophosphamide for 3 months and 80 mg prednisolone. I then switched to MMF (azathioprine made me sick all the time) which I'm still on, and my pred has tapered down to 5 mg. I still get a painful joint once every month or two. I treat it with naproxen (like a stronger ibuprofen) and it goes after about 36 hours. The rest of the time I can cycle, work and be happy. I'm on antidepressants to stop me getting gloomy - I got very down at one stage, but prayer, hope, and my family kept me going. The outlook for sufferers of this disease is now much better than twenty years ago, and I send my wishes to your daughter, there is light at the end of the tunnel. I have a superb rheumatologist in Haywards Heath. Glad to offer more advice if you need it. Best wishes.

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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.

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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

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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

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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.

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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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