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Granulomatosis with Polyangiitis

Medically reviewed by Last updated on Dec 4, 2023.

What is granulomatosis with polyangiitis (GPA)?

GPA is a rare blood vessel disease where your blood vessels are inflamed. Small lumps called granulomas may also form when the cells lining your blood vessels die. This can cause a decrease in blood flow to your organs, most commonly your respiratory tract, lungs, and kidneys.

What may cause GPA?

The exact cause is not known. Healthcare providers think that an abnormal immune response, such as an infection, may lead to GPA.

What are the signs and symptoms of GPA?

You may have any of the following:

  • A cough or coughing up blood
  • Blood in your urine or decreased urination
  • Body aches and fever
  • Ear and sinus infections
  • Red, swollen, burning, or painful eyes
  • Runny, sore nose and nosebleeds
  • Shortness of breath and chest pain
  • Skin and mouth sores

How is GPA diagnosed?

  • Blood and urine tests are done to check for infection, kidney function, and antibodies to GPA.
  • A chest x-ray checks for lung damage that may be caused by GPA.
  • A biopsy may be done to check for damage caused by GPA. A tissue sample from your affected organ is taken and sent to a lab for tests.

How is GPA treated?

  • Steroids are given to decrease inflammation.
  • Immunosuppressive therapy may be given to slow your immune system and help prevent organ damage.
  • Plasma exchange is done to separate the plasma in your blood from your blood cells. Plasma is the liquid part of your blood. Your blood cells are then returned to your body and your body creates new plasma. Plasma exchange may be needed if you have life-threatening organ damage. Ask for more information about plasma exchange.
  • Surgery may be needed to help control nosebleeds or repair damage to your nose. You may also need surgery to repair damaged tissue in your airway. A kidney transplant may be needed if GPA causes your kidneys to fail. Ask for more information about surgeries to treat the effects of GPA.

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

View more treatment options

Call your local emergency number (911 in the US) if:

  • You have sudden chest pain or shortness of breath.

Call your doctor if:

  • You have blood in your urine or are urinating less than normal.
  • You have new or increased hearing loss.
  • You have worse vision or vision loss.
  • You have a fever.
  • You have new sores on your skin or in your mouth.
  • You have red and swollen eyes.
  • You have questions or concerns about your condition or care.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.