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Granulomatosis with Polyangiitis

What is granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is an autoimmune disorder. An autoimmune disorder occurs when the body's immune system attacks and destroys healthy tissue. This condition causes inflammation, swelling, and irritation in blood vessels and other tissues.

This inflammation reduces or stops the flow of blood to organs in the body. The condition mostly affects the respiratory system. This includes the sinuses, nose, windpipe, and lungs. It can also affect the kidneys. But it can damage any organ in the body.

What causes granulomatosis with polyangiitis?

GPA is not common. It can start at any age. Doctors don't know what causes it.

What are the symptoms of granulomatosis with polyangiitis?

Most people with GPA first have vague symptoms that may include:

  • Tiredness or exhaustion
  • Joint pain
  • Upper respiratory symptoms that don’t respond to treatments for allergies or colds:
    • Runny nose
    • Bloody discharge
    • Sinus pain
    • Congestion
    • Blockage of the eustachian tubes in the ears
    • Cough
    • Cough that produces blood
    • Shortness of breath
  • Eye infections, redness, burning, or pain
  • Weakness
  • Skin sores or ulcers
  • Hoarseness
  • Fever
  • Night sweats

How is granulomatosis with polyangiitis diagnosed?

To diagnose GPA, your healthcare provider may do the following:

  • Take your health history
  • Do a physical exam
  • Review of your symptoms
  • Order blood tests
  • Order an antineutrophil cytoplasmic antibody (ANCA) blood test
  • Order imaging tests, such as chest X-rays or CT scans of the lungs or sinus
  • Do a biopsy from an affected organ to see if the condition is present
  • Order urine tests

Some of these tests can help rule out other causes of your symptoms. They may not confirm the diagnosis. A biopsy is the only way to know for sure if it’s GPA.

How is granulomatosis with polyangiitis treated?

Most people with GPA can be treated. But the medicines used to treat this condition could have side effects of their own. Be sure to talk about these side effects with your healthcare provider.

These are common medicines used in treatment:

  • Corticosteroids. This steroid helps reduce inflammation. Long-term steroid use can affect your bone health, so your healthcare provider may also watch and treat changes in your bone density.
  • Rituximab. This is an antibody against certain immune cells (B-cells). These cells help cause the autoimmune state in GPA.
  • Cyclophosphamide . This is used to treat active GPA.
    • This medicine may be given to keep symptoms in remission for 1 to 2 years. 
    • This is an alternative to methotrexate.
    • This medicine may be given along with prednisone to treat GPA and bring about remission.
  • Antibiotics. Certain infections are more common among people with this condition. Antibiotics may be given to treat or prevent infection.

GPA may come back even after successful treatment. Continue to follow up with your healthcare provider, even when you are in remission.

What are the possible complications of granulomatosis with polyangiitis?

Ongoing GPA can cause:

  • Collapse of cartilage in the nose
  • Infections of sinuses and ears
  • Hearing loss
  • Kidney damage
  • Kidney failure
  • Death

When should I call my healthcare provider?

Call your healthcare provider if you have the symptoms listed above. This is especially true if you have tried over-the-counter treatments that seem appropriate, such as allergy medicines, with no success.

Always call your healthcare provider if you have changes in vision. Call if you have a cough that produces bloody mucus. These may be symptoms of granulomatosis with polyangiitis (GPA) or another serious health problem.

Key points about granulomatosis with polyangiitis

  • Granulomatosis with polyangiitis (GPA) is an autoimmune disorder that causes swelling and irritation in blood vessels and other tissues. It is uncommon.
  • Doctors don't know what causes it.
  • Most people with GPA first report vague symptoms.
  • Biopsy is the only way to know for sure if it’s GPA.
  • Most people with GPA will find relief by taking strong medicines.
  • GGPA may come back even after successful treatment.
  • Ongoing GPA can have serious complications.
  • Continue to follow up with your healthcare provider, even when you are in remission.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.
Online Medical Reviewer: Daphne Pierce-Smith RN MSN CCRC
Online Medical Reviewer: Frederic Little MD
Online Medical Reviewer: Rita Sather RN
Date Last Reviewed: 8/1/2018
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