Diagnosis
Diagnosis involves the steps that your healthcare team takes to find out if you have granulomatosis with polyangiitis (GPA). Your healthcare professional asks you about your symptoms and health history. You also get a physical exam. You may need other tests as well.
Lab tests
Blood tests can check for:
- Signs of the inflammation that GPA causes. A high level of a protein made by the liver, called C-reactive protein, can be a clue for inflammation. So can red blood cells that quickly sink to the bottom of a test tube. This measurement is known as a high erythrocyte sedimentation rate, also called a high sed rate.
- Antineutrophil cytoplasmic antibodies. These immune system proteins attack healthy white blood cells called neutrophils by mistake. The antibodies appear in the blood of most people who have GPA.
- Low levels of healthy red blood cells to carry oxygen. This is called anemia, and it's common in people with GPA.
- Symptoms that the kidneys aren't properly filtering waste products from the blood.
Urine tests can reveal if the urine has red blood cells or too much protein. These might mean that the disease is affecting the kidneys.
Imaging tests
If you have lung symptoms, chest X-rays and CT scans can help find out if granulomatosis with polyangiitis is the cause. CT scans use X-rays and a computer to make more-detailed images of the organs inside the body. CT scans also can help find out if granulomatosis with polyangiitis is the cause of head or neck symptoms. Once a person starts treatment for GPA, healthcare professionals can use CT scans to find out if treatment is working.
Biopsy
During a biopsy, a healthcare professional removes a small sample of tissue from the affected area of the body. For example, a tissue sample could be taken from the lungs, skin, kidneys or inside of the nose. The tissue sample is checked with a microscope to look for signs of inflammation or damage. This test can confirm if you have granulomatosis with polyangiitis.
Treatment
Treatment for granulomatosis with polyangiitis (GPA) includes medicines to get the disease under control and keep it from coming back. Along with getting care from your regular healthcare professional, you'll likely need treatment and follow-up care from various specialists. The specialists that you need depend on the organs that the disease affects. With early treatment, many people who have GPA get better and lead full lives.
Medications
Medicines called corticosteroids help lessen the immune system response and lower inflammation of the blood vessels. Common side effects include weight gain, risk of infection and thinning of bones.
Other medicines that lessen the immune system response include:
- Rituximab (Rituxan).
- Azathioprine (Azasan, Imuran).
- Mycophenolate (CellCept, Myhibbin).
- Methotrexate (Trexall).
- Cyclophosphamide.
- Avacopan (Tavneos).
Once your condition is under control, you might stay on some medicines long-term. You might hear your healthcare professional call this maintenance therapy. The goal with long-term treatment is to prevent GPA from coming back, also called relapse. Medicines that can be used long-term include rituximab, methotrexate, azathioprine and mycophenolate.
Medicines such as these can raise the risk of infection. Cyclophosphamide can cause upset stomach, diarrhea and hair loss. Your healthcare professional may prescribe other medicines to help prevent side effects from prescribed treatments.
Plasma exchange
Plasma exchange removes the liquid portion of blood, called plasma. In most people with GPA, plasma has proteins in it that are linked with the disease. During plasma exchange, you receive fresh plasma or a protein made by the liver called albumin. Albumin helps the body make new plasma. Sometimes, plasma exchange is used to treat people who have very serious GPA. It can help the kidneys get better. Another name for this treatment is plasmapheresis.
Clinical trials
Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Coping and support
You're likely to get better after treatment for granulomatosis with polyangiitis (GPA). Even so, you might feel stress about the disease coming back or the damage it can cause. Here are some tips to help you cope:
- Understand your condition. Learn all that you can about GPA. The knowledge may help you deal with complications, medicine side effects and relapses. Talk with your healthcare professional. You also might want to talk with a counselor or a medical social worker.
- Build a strong support system. Family and friends can help you cope. And you might find it helpful and comforting to talk with other people who are living with the condition. Ask a member of your healthcare team about how to connect with a support group.
Preparing for your appointment
You're likely to start by seeing your usual healthcare professional. You also might be referred to a specialist such as:
- A doctor called a rheumatologist who treats conditions of the joints, muscles, and immune system.
- A doctor called a pulmonologist who treats lung conditions.
- A doctor called an otolaryngologists who treats ear, nose, and throat conditions.
- A kidney doctor called a nephrologist.
- A doctor called a neurologist who treats conditions of the nervous system.
A specialist likely will be the one to find out if you have granulomatosis with polyangiitis.
Here's some information to help you get ready for your appointment.
What you can do
Be aware of any rules to follow before your healthcare checkup. When you make the appointment, ask if you need to do anything in advance. For instance, you may be told to limit your diet.
If you've had any recent blood tests or chest X-rays at another medical office or hospital, ask the staff there to forward the test results and X-rays to the healthcare professional you're seeing. Or pick up the material yourself to be sure it gets to your healthcare professional.
Get a referral if your insurance company requires it for visits to specialists. Be sure a letter of referral has been sent to the healthcare professional or bring the letter with you.
Before your appointment, also make a list of:
- Your symptoms and when they began.
- Key personal medical information, including other recent health conditions and major stresses you've had.
- Medicines, vitamins and supplements you take, including doses.
- Questions to ask your healthcare professional.
Take a family member or friend with you to the appointment if you can. This person can help you remember the information you receive.
For granulomatosis with polyangiitis, questions to ask your healthcare team 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 short-term or long-term?
- What treatment do you recommend?
- Are there any treatment choices aside from the main treatment that you're suggesting?
- How long will treatment last?
- I have another medical condition. How can I best manage my 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 healthcare professional is likely to ask questions such as:
- Do your symptoms happen often or just once in a while?
- How bad are your symptoms?
- Have you had a fever?
- What, if anything, seems to make your symptoms better?
- What, if anything, appears to make your symptoms worse?
- Do you smoke?
What you can do in the meantime
If your symptoms are getting worse, tell your main healthcare professional. That way, your healthcare professional can try to get you to a specialist quickly.
Jan. 14, 2025