Granulomatosis with polyangiiti

NHS Choices information on granulomatosis with polyangiitis (Wegener's granulomatosis), with links to other useful resources.

Information written and reviewed by Certified Doctors.

Contents

Key Information

What should I do?

If you think you have this condition you should see a doctor within 48 hours.

How is it diagnosed?

Your doctor might suspect Wegener’s Granulomatosis based on your symptoms and examination. You might be referred to a specialist, who may recommend blood tests or analysis of a small sample (biopsy) of one of your blood vessels. Body imaging using computerised tomography (CT) or magnetic resonance imaging (MRI) scans may be used to check whether the condition has affected any other organs besides your blood vessels.

What is the treatment?

If you are diagnosed with this condition, and it is currently active, it is important to bring it under control (achieve ‘remission’). This is done by taking high-dose steroid medication for a short period of time.

Once your condition is in remission, you might be prescribed medications to be taken over the longer term:

  • immunosuppressant medications to prevent flare-ups from occurring
  • antibiotics to prevent infections.

Introduction

Granulomatosis with polyangiitis (doctorA) 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.

doctorA 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 doctorA 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, doctorA can lead to tissue damage and organ failure.

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

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

What are the symptoms?

Typical symptoms of doctorA are:

  • a persistent unexplained fever
  • night sweats
  • fatigue and weakness
  • a general ill feeling
  • loss of appetite and weight loss
  • joint pain and soreness
  • persistent ear infections and reduced hearing
  • frequent sinusitis
  • recurrent nosebleeds and sores around the nose
  • chest pain, cough or shortness of breath
  • coughing up blood or blood in the phlegm
  • blood in the urine

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

Who is affected?

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 doctorA is unknown. It is thought that doctorA 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 doctorA have a gene that makes them more likely to develop the condition. doctorA 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 doctorA to occur in two people in the same family.

How is it diagnosed?

Your doctor may suspect doctorA 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 doctorA uses to attack the body's own blood vessels. However, this test can sometimes come back negative in people with doctorA, 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 doctorA, 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 doctorA 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 doctorA may also need antibiotics to take over a long period, to help prevent infections.

A small group of people with doctorA 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 doctorA.

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 doctorA will have a relapse (the disease returns). This usually happens within two years of stopping treatment.

Most people with doctorA lead normal lives. If you have doctorA 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 doctorA 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.

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