What is granulomatosis with polyangiitis (GPA, formerly called Wegener’s)?

Granulomatosis with polyangiitis (GPA, formerly called Wegener’s) is a rare disease of uncertain cause. It is the result of inflammation within the tissues called granulomatous inflammation and blood vessel inflammation ("vasculitis"), which can damage organ systems. The areas most commonly affected by GPA include the sinuses, lungs, and kidneys, but any site can be affected.

Inflamed blood vessel

What is vasculitis?

Vasculitis is a general term that refers to inflammation of the blood vessels. When inflamed, the blood vessel may become weakened and stretch forming an aneurysm, or become so thin that it ruptures resulting in bleeding into the tissue. Vasculitis can also cause blood vessel narrowing to the point of closing off the vessel entirely. This can cause organs to become damaged from loss of oxygen and nutrients that were being supplied by the blood.

Vasculitis

What are the features of granulomatosis with polyangiitis (GPA)?

GPA primarily affects the upper respiratory tract (sinuses, nose, trachea [upper air tube]), lungs, and kidneys. Any other organ in the body can be affected as well.

Granulomatosis with polyangiitis affected sites

The symptoms of GPA and their severity vary among patients. General signs of the disease may include:

  • Loss of appetite
  • Weight loss
  • Fever
  • Fatigue

Most patients first notice symptoms in the respiratory tract. Symptoms may include:

  • Persistent runny nose (also called rhinorrhea) or the formation of nasal crusts and sores
  • Nasal or facial pain
  • Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
  • Cough that might include bloody phlegm caused by upper airway or lower airway (lung) inflammation
  • Chest discomfort with or without shortness of breath
  • Middle ear inflammation (also called otitis media), pain, or hearing loss
  • Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Other possible features include:

  • Eye inflammation and/or pressure behind the eye, making eye movement difficult, with or without loss of vision
  • Joint pain (arthritis) or muscle pain
  • Rashes or skin sores
  • Kidney inflammation (although kidney inflammation is common, it is not usually associated with symptoms, such as pain).

Who is affected by granulomatosis with polyangiitis (GPA)?

GPA can occur in people of all ages. The peak age groups affected are from 40-60 years. It appears to affect men and women equally.

What causes granulomatosis with polyangiitis (GPA)?

The cause of GPA is unknown. GPA is not a form of cancer, it is not contagious, and it does not usually occur within families. Evidence from research laboratories strongly supports the idea that the immune system plays a critical role in GPA such that the immune system causes blood vessel and tissue inflammation and damage.

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