October 6, 2015
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Granulomatosis with Polyangiitis (GPA or Wegener's Granulomatosis)

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Granulomatosis with Polyangiitis (GPA or Wegener's Granulomatosis) facts

  • Granulomatosis with polyangiitis (GPA or Wegener's granulomatosis) is an uncommon disease that involves inflammation of blood vessels (vasculitis).
  • Symptoms of GPA include:
    • fatigue,
    • weight loss,
    • fevers,
    • shortness of breath,
    • bloody sputum,
    • joint pains, and
    • sinus inflammation.
  • Diagnosis of GPA is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis.
  • Treatment is directed toward stopping the inflammation process by suppressing the immune system.

What is granulomatosis with polyangiitis (GPA)?

Granulomatosis with polyangiitis (GPA) is an uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages(sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized granulomatosis with polyangiitis. When only the lungs are involved, the condition is sometimes referred to as limited granulomatosis with polyangiitis (GPA). Doctors typically avoid the older name "Wegener's granulomatosis" because the German physician Friedrich Wegener, the disease's former namesake, was a member of the Nazi party and allegedly linked to Nazi war crimes. The terminology "granulomatosis with polyangiitis" highlights the two central pathologic features of the disease, granuloma formation and inflammation of blood vessels (angiitis or vasculitis).

GPA usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of GPA is not known.

Medically Reviewed by a Doctor on 5/20/2015

Source: MedicineNet.com


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