Granulomatosis with Polyangiitis (GPA) is a rare disease that is a form of vasculitis or inflamed blood vessels of the nose, throat, sinuses, lungs, and kidneys. First described in 1931 by a German medical student named Heinz Klinger, Granulomatosis with Polyangiitis usually affects people between the ages of 40 to 65. Granulomatosis with Polyangiitis is a severe disease and can lead to death if left untreated. It may appear suddenly, or it may develop over several months.
You may occasionally hear GPA called Wegener's granulomatosis. This is because the disease was initially called Wegener’s arteritis, Wegener’s disease, or Wegener's granulomatosis. It was named after a German pathologist, Friedrich Wegener, who had three cases and recognized it as a different form of vasculitis. The name was changed to Granulomatosis with Polyangiitis or even GPA to remove any association with Wegener who was a Nazi war criminal.
Granulomatosis with Polyangiitis can affect several parts of your body. It commonly affects the respiratory system, including:
It can also affect other parts of your body including:
Sometimes it will only affect one part of your body, such as just the lungs or kidneys.
Granulomatosis with Polyangiitis has a number of common symptoms. They include:
Granulomatosis with Polyangiitis can cause the following complications without treatment:
Some of these complications can be life-threatening, which is why if you have any of the above symptoms mentioned, you should see a doctor about getting treated.
Granulomatosis with Polyangiitis in the inflammation of the arteries and veins. It typically affects the arteries that provide a blood supply to the kidneys, lungs, and sinuses. Sometimes it only affects one organ, such as the lungs. This is an incomplete form of the disease. The inflammation is in the form of granulomas that can severely damage the organs.
Unfortunately, doctors do not know what precisely causes Granulomatosis with Polyangiitis. They know that usually occurs after an infection, but no particular infection is a culprit. There may or may not be a small genetic component to it, but it is rare for two members of the same family to have it. Although it typically affects people between the ages of 40 and 65, it can affect anyone at any age.
Your doctor will examine your and go through your symptoms. If they suspect Granulomatosis with Polyangiitis, they will need to run several tests to determine if you have the disease. This includes the biopsy of the trachea, sinuses, nose, and ears. Not as invasive but is less than 50 percent successful at diagnosis.
The best way to prove there is Granulomatosis with Polyangiitis is a lung biopsy, although a kidney biopsy may be used as well. These biopsies, while invasive, are usually able to confirm the diagnosis. The lung biopsy may be done thoracoscopic or open. The kidney biopsy usually requires a smaller tissue sample.
There are several less invasive ways to diagnose Granulomatosis with Polyangiitis. These include:
Treatment of Granulomatosis with Polyangiitis is aimed at reducing inflammation and suppression of the immune system which reacts to the inflammation with more inflammation. Medications include:
About 90 percent of patients respond to treatment with 75 percent having remission. However, there is a high relapse rate which means that those who have Granulomatosis with Polyangiitis will need to continue with medications to prevent relapse.
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