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Granulomatosis with polyangiitis

The condition granulomatosis with polyangiitis is quite rare – altogether there are only 1,000 new cases of granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis a year in the UK. It's slightly more common in men than in women.

It usually develops with ear, nose and throat problems, including nosebleeds and crusting of the nose, and occasionally coughing up blood (haemoptysis). These symptoms can appear a year or two before more general vasculitis starts.

This general vasculitis usually involves the skin, lungs, eyes and kidneys. The kidney problems can sometimes lead to kidney failure if they're not recognised early.

The name of this disease has changed recently. It was previously called Wegener's granulomatosis after the doctor who was thought to have discovered it. The new name describes the features of the disease:

  • polyangiitis means that many (poly = many) arteries are involved
  • granulomatosis means that when a biopsy of the inflamed tissue is studied under a microscope you can see swellings called granulomata.

Remember that the terms Wegener's granulomatosis, granulomatiosis with polyangiitis, and GPA describe exactly the same disease.

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