Granulomatosis with polyangiitis (GPA)
- Medically reviewed by
- AKF's Medical Advisory Committee
- Last updated
- December 20, 2022
What is granulomatosis with polyangiitis (GPA)?
GPA is a rare disease that causes swelling (inflammation) and irritation in your blood vessels. Areas of inflammation, called granulomas, make it harder for blood to get to the organs and tissues that need it, which can cause damage.
Damage from GPA can affect your:
- Lungs and trachea (the tube that connects your lungs to your nose and mouth)
- Sinuses (the air-filled spaces behind your cheeks and forehead that open into your nose)
GPA is a rare but serious disease that can get worse quickly and cause kidney failure, lung failure or death if it is not found early and treated.
GPA used to be called Wegener's granulomatosis.
What are the symptoms of GPA?
The first symptoms of GPA are usually in your nose, sinuses, throat or lungs. You may notice:
- A runny nose that will not stop
- Sores or crusty buildup in your nose
- Sinus infections
Other symptoms may include:
- Pain in your nose or the area around your nose and eyes
- Feeling weak and tired
- Coughing, including coughing up blood
- Trouble catching your breath or wheezing
- Pain or discomfort in your chest
- Feeling achy
- Numbness in your arms, legs, fingers or toes
- Pain in your ear or hearing loss
- Sudden loss of vision
- Loss of appetite or weight loss
- Joint or muscle pain
- Rashes or sores on your skin
How does GPA affect the kidneys?
GPA can damage your kidneys by lowering the amount of blood that reaches them. This can lower your kidneys' ability to filter waste and fluid out of your blood. GPA can cause kidney failure if it is not treated. If your kidneys fail, you will need dialysis or a kidney transplant.
What causes GPA?
Doctors do not know exactly what causes GPA, but they believe your body's immune system causes the inflammation in your blood vessels.
GPA is not contagious and doctors do not think it is passed down through your family (inherited).
GPA can affect anyone at any age, but it is most common in people ages 40–65 and people of European descent.
How do doctors treat GPA?
If you have GPA, doctors may use urine tests to keep track of your kidney function. Doctors treat GPA with medicines that:
- Lower or stop your immune system's response (immunosuppressants)
- Lower inflammation (corticosterioids and glucocorticoids)
During treatment, doctors keep track of your symptoms and the amount of inflammation in your blood vessels to see how well the treatment is working. For some patients, treatment can lead to having very low or no inflammation or symptoms from GPA (remission).
If GPA is in remission for at least two years, doctors may begin to lower your dose of medicines slowly. But even after going into remission for years, GPA can come back (relapse) and you will need to start treatment again.
When GPA is found early, it can be successfully treated. More than 7 out of 10 (75%) of patients reach remission. Eight out of 10 (80%) patients are still alive eight years after their diagnosis. Research has shown that new medicines called anti-IL-5 therapy can help patients stay in remission.
What kind of doctor treats GPA?
Because GPA affects different areas of your body, patients with GPA are treated by a team of specialists, such as:
- Rheumatologists (doctors that treat the immune system and diseases of the muscles and bones, such as arthritis)
- Pulmonologists (lung and respiratory system doctors)
- Nephrologists (kidney doctors)
- Otolaryngologists (ear, nose and throat doctors)
How will I know if I have GPA?
Because many symptoms of GPA are similar to those caused by other health problems, doctors use a variety of tests and exams together to find out if you have GPA, including:
- A physical exam
- Imaging tests, such as an X-ray or CT scan
- Taking out part of the tissue to look at it under a microscope (biopsy)
- Blood tests
How can I prevent GPA?
Because doctors do not know the exact cause of GPA, it is not clear how to prevent it.