Diagnosis

Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history.

Lab tests

Blood tests can check for:

  • Signs of inflammation, such as a high level of C-reactive protein or a high erythrocyte sedimentation rate — commonly referred to as a sed rate.
  • Anti-neutrophil cytoplasmic antibodies, which appear in the blood of most people who have active granulomatosis with polyangiitis.
  • Anemia, which is common in people with this disease.
  • Signs that your kidneys aren’t properly filtering waste products from your blood.

Urine tests can reveal whether your urine contains red blood cells or has too much protein, which might indicate that the disease is affecting your kidneys.

Imaging tests

Chest X-rays, CT or MRI can help determine which blood vessels and organs are affected. They can also help your doctor monitor whether you're responding to treatment.

Biopsy

This is a surgical procedure in which your doctor removes a small sample of tissue from the affected area of your body. A biopsy can confirm a diagnosis of granulomatosis with polyangiitis.

Treatment

With early diagnosis and appropriate treatment, you might recover from granulomatosis with polyangiitis within a few months. Treatment might involve taking prescription drugs long term to prevent relapse. Even if you're able to stop treatment, you'll need to regularly see your doctor — and possibly several doctors, depending on which organs are affected — to monitor your condition.

Medications

Corticosteroids such as prednisone help suppress the immune system and reduce inflammation of the blood vessels. Common side effects include weight gain, risk of infection and osteoporosis.

Other drugs that suppress your immune system include cyclophosphamide, azathioprine (Azasan, Imuran), mycophenolate (CellCept) and methotrexate (Trexall). Rituximab (Rituxan) is another option for treating granulomatosis with polyangiitis. It's given by injection, and often is combined with corticosteroids.

Once your condition is controlled, you might remain on some drugs long term to prevent relapse. These include rituximab, methotrexate, azathioprine and mycophenolate.

Side effects of immune-suppressing drugs include increased risk of infection. Cyclophosphamide can cause nausea, diarrhea and hair loss. Your doctor may prescribe other drugs to help prevent side effects from prescribed treatments.

Plasma exchange

Also known as plasmapheresis, this treatment removes the liquid portion of your blood (plasma) that contains disease-producing substances. You receive fresh plasma or a protein made by the liver (albumin), which allows your body to produce new plasma. In people who have very serious granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.

More Information

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.

Coping and support

With treatment you're likely to recover from granulomatosis with polyangiitis. Even so you might feel stress about possible relapse or damage the disease can cause. Here are some suggestions for coping:

  • Understand your condition. Learning about granulomatosis with polyangiitis might help you deal with complications, medication side effects and relapses. Besides talking with your doctor, you might want to consult a counselor or a medical social worker.
  • Maintain a strong support system. Family and friends can help you cope. And you might find it informative and comforting to talk with other people who are living with the condition. Ask a member of your health care team about connecting with a support group.

Preparing for your appointment

You're likely to start by seeing your primary care doctor, who then might refer you to a specialist in the lungs (pulmonologist); ear, nose and throat (otolaryngologist); kidneys (nephrologist); or bones and joints (rheumatologist). A specialist will likely be the one to diagnose you.

Here's some information to help you get ready for your appointment.

What you can do

Be aware of pre-appointment restrictions. When you make the appointment, ask if you need to do anything in advance, such as restrict your diet.

If you've had any recent blood tests or chest X-rays at another medical office or hospital, ask the staff to forward the test results and X-rays to the doctor you're seeing. Or pick up the material yourself to be sure it gets to your doctor.

Get a referral if your insurance company requires it for visits to specialists. Be sure a letter of referral has been sent to the doctor, or bring it with you.

Make a list of:

  • Your symptoms and when they began
  • Key personal medical information, including other recent health problems and major stresses you've had
  • Medications, vitamins and supplements you take, including doses
  • Questions to ask your doctor

Consider taking a family member or friend with you to the appointment to help you remember the information you receive.

For granulomatosis with polyangiitis, questions to ask your doctor include:

  • What is likely causing my symptoms? What are other possible causes?
  • What tests will I need? How do I prepare for them?
  • Is my condition temporary?
  • What treatment do you recommend?
  • What are alternatives to the primary approach that you're suggesting?
  • How long will treatment last?
  • I have another medical condition. How can I best manage these conditions together?
  • Do you have brochures or other printed materials to give me? What websites do you recommend?

What to expect from your doctor

Your doctor is likely to ask questions, including:

  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • Have you been running a fever?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do you smoke?

What you can do in the meantime

If your symptoms are worsening, tell your primary care doctor so that he or she can try to get you to a specialist quickly.

Dec. 20, 2018
  1. AskMayoExpert. Granulomatosis with polyangiitis. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2018.
  2. Granulomatosis with polyangiitis (Wegener's). American College of Rheumatology. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Granulomatosis-with-Polyangitis-Wegners. Accessed Nov. 20, 2018.
  3. Granulomatosis with polyangiitis (Wegener's). Vasculitis Foundation. https://www.vasculitisfoundation.org/education/granulomatosis-with-polyangiitis-gpa-wegeners/. Accessed Nov. 20, 2018,
  4. Falk RJ, et al. Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis. https://www.uptodate.com/contents/search. Accessed Nov. 20, 2018.
  5. Wojciechowska J, et al. Granulomatosis with polyangiitis in otolaryngologist practice: A review of current knowledge. Clinical and Experimental Otolaryngology. 2016;9:8.

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