What is Wegener’s granulomatosis or granulomatosis with polyangiitis, what are the symptoms and how’s it treated?

GRANULOMATOSIS with polyangiitis is a serious condition that can have life-threatening consequences if it is left untreated.

But what are the symptoms to look out for and how can you test for it?

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What is Wegener's granulomatosis or granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (GPA), which used to be known as Wegener's granulomatosis, is a rare condition where the blood vessels become inflamed.

Typically it affects older or middle-aged people, but all ages can have symptoms, even children.

Mostly GPA affects the ears, nose, kidneys, lungs and sinuses.

The condition can be serious, but with medication, sufferers can live relatively normal lives.

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The cause of GPA is unknown but it thought to be linked to an issue with the immune system.

What are the symptoms of granulomatosis with polyangiitis?

It depends what part of the body is affected as to what symptoms are present, but these can include:

• tiredness, high temperature, loss of appetite, weakness, weightloss and joint pain
• lung problems, chest pain, wheezing and shortness of breath
• kidney problems like blood in urine, high blood pressure, kidney inflammation
• rashes, purple spots or lumps on the skin
• ear, nose or throat problems like hearing loss, blocked nose or earache
• irritated eyes or double vision
• stomach pain, diarrhoea or blood in faeces

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How is granulomatosis with polyangiitis tested?

Diagnosing someone with GPA can be hard as the symptoms are often the same as a number of different conditions.

An individual may require multiple tests before a final diagnosis is made.

The tests can include:

• monitoring symptoms
• examining affected areas
• urine samples to test kidney efficiency
• blood tests to look for ANCAs (antineutrophil cytoplasmic antibodies) which are thought to be involved with GPA
• having a biopsy from an affected area to check for inflammation
• An X-ray, CT scan or MRI scan of the affected area

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How is granulomatosis with polyangiitis treated?

The condition is thought to be linked to the immune system so is typically treated with medicines that reduce the activity of the immune system.

The three stages of treatment include:

Getting control of the condition

This can be done by taking daily tablets or having injections of a medicine called cyclophosphamide every two or three weeks.

It could also involve daily steroid tablets or having steroid injections alongside cyclophosphamide injections.

You must discuss all treatment with your doctor so you are aware of any potential side effects.

Some individuals require a plasma exchange, and their blood is filtered via a machine that removes any antibodies linked to GPA.

It can take a few months to get the condition under control.

Maintaining control

You must be diligent to prevent the symptoms from returning once you have control.

For two to five years, patients will typically:

• stop cyclophosphamide treatment
• start taking a less powerful medicine such as methotrexate or azathioprine
• taking daily steroids tablets

Treating symptoms

You may need a different treatment if your symptoms return.

This can include increasing dosages of steroids or having more cyclophosphamide injections.

You may also need a course of plasma exchange or treatment with rituximab.