blood cells

There are 3 different types of ANCA-associated vasculitis;

  • Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis
  • Microscopic polyangiitis (MPA)
  • Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome

These three conditions are grouped together under the umbrella term ‘ANCA-associated vasculitis’ because they are all associated with a key protein that is found in the blood, called ‘ANCA’, and, they all cause inflammation or damage to small blood vessels.  These small blood vessels are found all over the body, so any part of the body can be affected, but most commonly the kidneys, lungs, joints, ears, nose, and nerves are affected.

Photo of lady coughing

What are the Symptoms?

Non-specific symptoms are most common, which makes diagnosis difficult.  These include

  • fatigue
  • weight loss
  • unexplained fever
  • night sweats
  • muscle and/or joint pain

More specific symptoms may include

  • Worsening shortness of breath or cough, sometimes with coughing blood
  • a non-blanching rash
  • earache or hearing loss
  • drop in kidney function (found on blood tests)

Blood tests for the ANCA protein will also be requested if an ANCA associated vasculitis is suspected.

Who is affected?

Vasculitis affects men and woman equally, and across all ethnicities.

It can affect people at any age, but is very rare in childhood; incidence rises with age.  There is an estimated 12000 cases of ANCA vasculitis in the UK, although this number is likely to be higher.

 Why does it happen?

There is evidence that both genetics and environmental exposures contribute to the development of ANCA associated vasculitis.  However, why ANCA associated vasculitis may develop in different people at different ages is poorly understood.  Infections has been suggested as one possible trigger.

Man with an oxygen mask on


ANCA associated vasculitis can be managed by different specialities such as rheumatology, renal, respiratory, or dedicated vasculitis service.

It is typically initially treated with prednisolone (steroids) to control the inflammation.  Other medications which are likely to be used alongside steroids include cyclophosphamide, rituximab, azathioprine, methotrexate, and mycophenolate mofetil.  By clicking on the drug names, you will be taken to the Versus Arthritis website, where you can read about each specific medication.

For more information and support on ANCA associate vasculitis visit – Vasculitis UK
For more general information on any of the conditions or medications mentioned above, you may find visiting these websites useful:

Versus Arthritis

NHS Website