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Wegener’s Granulomatosis

Wegener’s Granulomatosis

What is Wegener’s granulomatosis?

Wegener’s granulomatosis, now called granulomatosis with polyangiitis, is a condition of the immune system. It causes swelling and irritation in blood vessels and other tissues.

This inflammation cuts down 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. However, it can damage any organ in the body.

What causes Wegener’s granulomatosis?

Wegener’s granulomatosis is not common. It can start at any age. The cause of Wegener’s granulomatosis is not known.

What are the symptoms of Wegener’s granulomatosis?

Most people with Wegener’s granulomatosis 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 Wegener’s granulomatosis diagnosed?

To diagnose this condition, your healthcare provider may do the following:
  • Taking your medical history
  • Physical exam
  • Review of your symptoms
  • Blood tests
  • Antineutrophil cytoplasmic antibody (ANCA) blood test
  • Imaging tests, such as chest X-rays or CT scans of the lungs or sinus
  • Biopsy from an affected organ to see whether the condition is present
  • Urine tests

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

 

How is Wegener’s granulomatosis treated?

Most people with Wegener’s granulomatosis can be treated. But the drugs 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.
  • Cyclophosphamide (also called Cytoxan). This is used to treat active Wegener’s granulomatosis.
    • This drug may be given to keep symptoms in remission for 1 to 2 years. 
    • This is an alternative to methotrexate.
    • This medicine may be given in combination with prednisone to treat Wegener’s granulomatosis and bring about remission.
  • Antibiotics. Certain infections are more common among people with this condition. Antibiotics may be given to treat or prevent infection.

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

What are the complications of Wegener’s granulomatosis?

Ongoing Wegener’s granulomatosis 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. Or call if you have a cough that produces bloody mucus. These may be symptoms of Wegener’s granulomatosis or another serious health problem.

Key points

Wegener’s granulomatosis is a condition of the immune system that causes swelling and irritation in blood vessels and other tissues. It is uncommon.

  • The cause of Wegener’s granulomatosis is unknown.
  • Most people with Wegener’s granulomatosis first report vague symptoms.
  • Biopsy is the only way to know for sure if it’s Wegener’s granulomatosis.
  • Most people with Wegener’s granulomatosis will find relief by taking strong medicines.
  • Wegener’s granulomatosis may come back even after successful treatment.
  • Ongoing Wegener’s granulomatosis can have serious complications.
  • Continue to follow up with your health care 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.

Disclaimer: This information is not intended to substitute or replace the professional medical advice you receive from your child's physician. The content provided on this page is for informational purposes only, and was not designed to diagnose or treat a health problem or disease. Please consult your child's physician with any questions or concerns you may have regarding a medical condition.