What is Granulomatosis with Polyangiitis?

Granulomatosis with Polyangiitis, previously known as Wegener’s granulomatosis, is a condition of the immune system that causes 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 most often affects the respiratory tract–the sinuses, nose, windpipe, and lungs–and the kidneys, but it can damage any organ in the body.

Granulomatosis with Polyangiitis is uncommon. It occurs in about one out of 20,000 to 30,000 people. It can strike at any age. The cause of Granulomatosis with Polyangiitis is still unknown.

Signs and symptoms

Most people with Granulomatosis with Polyangiitis first report 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


Ongoing Granulomatosis with Polyangiitis can result in:

  • Collapse of cartilage in the nose

  • Infections of sinuses and ears

  • Hearing loss

  • Kidney damage

  • Kidney failure

  • Death

When to call a doctor

Call your doctor if you are experiencing the symptoms listed above, especially if you have tried over-the-counter treatments that seem appropriate, such as allergy medications, with no success.

Always call your doctor if you have changes in vision or a cough that produces bloody mucus, which may be symptoms of Granulomatosis with Polyangiitis or another serious health condition.


Your doctor visit may include:

  • Taking your medical history

  • Physical exam

  • Review of your symptoms

  • Blood tests. These can’t diagnose Granulomatosis with Polyangiitis, but they can rule out other causes of your symptoms.

  • Antineurtophil cytoplasmic antibody (ANCA) blood test. In addition to routine blood tests, health care providers will often look for this specific antibody, but a positive or negative test does not confirm the diagnosis. 

  • Imaging tests, such as chest X-rays or CT scans of the lungs or sinus

  • Biopsy, or taking a sample of tissue from an affected organ to see whether the condition is present. This is the only way to know for sure if it’s Granulomatosis with Polyangiitis.

  • Urine tests


Most people with Granulomatosis with Polyangiitis will find relief. But the drugs used to treat this condition all have potential side effects of their own. Be sure to discuss these side effects in detail with your doctor.

These are common medications used in treatment:

  • Prednisone. This is a steroid that helps reduce inflammation. Long-term steroid use can affect your bone health, so your doctor may also monitor and treat changes in your bone density.

  • Cyclophosphamide (also called Cytoxan). This is used to treat active Granulomatosis with Polyangiitis.

  • Methotrexate. This drug may be given to keep symptoms in remission for one to two years. You may need to take folic acid supplements with this drug.

  • Azathioprine. This is an alternative to methotrexate.

  • Rituximab. This medication may be given in combination with prednisone to treat Granulomatosis with Polyangiitis and bring about remission.

  • Antibiotics (also called Imuran). Certain infections are more common among people with this condition. Antibiotics may be given to treat or prevent infection.

Granulomatosis with Polyangiitis may return even after successful treatment. Continue to follow up with your doctor, even when you are in remission.

To request an appointment with a physician at St. Louis Children's Hospital, call 314.454.5437 or 800.678.5437 or email us.