Wegener’s granulomatosis (WG) is a rare, long-term chronic condition that causes small blood vessels in the body to become inflamed.

This inflammation (called vasculitis) can affect several organs in the body such as the nose, lungs, eyes or kidneys.

What causes WG and who can get it?

No one knows exactly what causes WG. But we do know that the disease is not contagious – that means you can’t catch it off someone else or, if you have WG, give it to your family or friends. 

It is likely that white blood cells in the body, which usually help fight infection, instead cause damage to healthy cells.

Most people who get WG are adults but children can be affected too.

What are the symptoms and signs of WG?

The symptoms of WG vary between patients and depend on which organs have been affected.

In general, you may feel tired and feverish and you may have lost weight.

Problems with the skin, such are rashes, are common. Sometimes people also have painful joints and muscles.

Lots of people with WG first notice symptoms in their nose: nosebleeds, crusting and some pain. Some people can also have problems with their ears, with pain and some hearing loss.

If WG has affected your kidneys, doctors might find blood or protein in your urine, or you may have high blood pressure.

If the disease has affected your lungs, you may feel short of breath and cough up some blood.
Other parts of the body that can be affected include the wind-pipe, sinuses and eyes.

How is WG normally diagnosed?

The doctors will look at all your symptoms and do various tests, including blood tests, to find out if you have WG. This is because people with WG usually have an abnormal marker in their blood – an antibody called ANCA.

Normally, antibodies help fight infection. But ANCA antibodies attack healthy cells. Doctors will do a blood test to check for ANCA.

A chest x-ray can show whether the disease has affected your lungs.

Sometimes, a small tissue sample (usually from the kidney, lung or skin) will be taken and looked at under a microscope.

How is WG normally treated?

WG is treated using drugs that reduce inflammation and drugs that dampen the immune system (steroids and immunosuppressants).

At first, these drugs will be in high doses and you may have to have them through a drip or as an injection. Then you will have regular medications to take by mouth.

People with WG also take an antibiotic which has been shown to help prevent relapses and infections.
Unfortunately, some patients’ kidneys have been so badly damaged that they have to have dialysis.

Some people may also need plasma exchanges (blood filtering) to help get the disease under control.
Lots of research is being done into other drugs that might help treat WG. Your doctor will talk to you about these.

Looking forward

If you receive treatment early the outlook is reasonably good. Currently there is no cure for WG but the drugs can work well at controlling the disease and its symptoms.

Once your symptoms have improved, your doctor may try to reduce the amount of medication that you are taking. This will be done slowly over a long time.

Unfortunately, it is common for people with WG to have a relapse, where their symptoms return.
This is especially likely if you have started taking a lower dose of medication or have come off all medication. If you have a relapse the doctors will increase or change your medication.

Most patients lead normal lives but will need to be reviewed regularly at their doctors’ clinic to monitor the medications and their response to treatment; they will also be looking for signs of relapse.

For more help and information

• The Vasculitis Foundation is an international organization for patients with Vasculitis.
• The Stuart Strange Vasculitis Trust provide information and advice on all types of vasculitis, including Wegener’s granulomatosis.

Last reviewed by Great Ormond Street Hospital: 30 November 2009

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