Granulomatosis with polyangiitis
Medically reviewed on Aug 8, 2017
Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys.
Granulomatosis with polyangiitis, formerly called Wegener's granulomatosis, is one of a group of blood vessel disorders called vasculitis. It slows blood flow to some of your organs. The affected tissues may develop areas of inflammation called granulomas, which sometimes affect how these organs work.
Early diagnosis and treatment of granulomatosis with polyangiitis may lead to a full recovery. Without treatment, granulomatosis with polyangiitis can be fatal.
Signs and symptoms of granulomatosis with polyangiitis may develop suddenly or over several months. The first warning signs usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. The condition of people with this disease often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys.
Signs and symptoms of granulomatosis with polyangiitis may include:
- Runny nose, stuffiness, sinus infections and nosebleeds
- Coughing, sometimes with bloody phlegm
- Shortness of breath or wheezing
- Fatigue and general aches and pains
- Numbness in your limbs, fingers or toes
- Weight loss
- Blood in urine (hematuria)
- Skin sores or bruising
- Eye redness, burning or pain
- Ear infections
For some people, the disease affects only the lungs. When the kidneys are affected, you may not notice any early warning signs. But blood and urine tests can detect the problem. Without treatment, kidney failure and anemia often occur.
When to see a doctor
See your doctor if you have a runny nose that doesn't respond to over-the-counter cold medicines, especially if it's accompanied by nosebleeds and pus-like material, coughing up blood, or other warning signs of granulomatosis with polyangiitis. Because this disease can get worse quickly, early diagnosis is key to getting effective treatment.
No one knows exactly what causes granulomatosis with polyangiitis. It appears to develop after an infection or other inflammation-causing event triggers an abnormal reaction from your immune system.
This reaction can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas). Granulomas can destroy normal tissue, and narrowed blood vessels reduce the amount of blood and oxygen that reaches your body's tissues and organs.
Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65.
Besides affecting your nose, throat and lungs, granulomatosis with polyangiitis may affect your skin, eyes, ears, kidneys, heart and other organs. Complications may include:
- Hearing loss
- Skin scarring
- Heart disease
- Kidney damage
- A loss of height in the bridge of the nose (saddling) caused by weakened cartilage
- Deep vein thrombosis
Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history. He or she may also have you undergo diagnostic tests and procedures, such as the following:
Blood tests. These tests can be used to look 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. A blood test that looks for certain antibodies — the anti-neutrophil cytoplasmic antibodies test — may be helpful because these substances appear in the blood of most people who have active granulomatosis with polyangiitis.
Another blood test can check for anemia, which is common in people with this disease. A blood test for creatinine can check whether your kidneys are properly filtering waste products from your blood.
- Urine tests. These tests may reveal whether your urine contains red blood cells or has too much protein, which may indicate the disease is affecting your kidneys.
- Chest imaging. Imaging tests can help determine what blood vessels and organs are affected. They can also help your doctor monitor whether you're responding to treatment. You may be asked to undergo chest X-rays, computerized tomography (CT) or magnetic resonance imaging (MRI).
- Biopsy. This is a surgical procedure in which your doctor removes a small sample of tissue from the affected area of your body. It's the only way to confirm a diagnosis of granulomatosis with polyangiitis.
With early diagnosis and appropriate treatment, you may recover from granulomatosis with polyangiitis within a few months. You may need to keep taking prescription drugs long term to prevent relapse. Even if you're able to stop treatment, you'll need to see your doctor regularly so that he or she can continue to monitor your condition closely.
Talk with your doctor about the drug or drug combinations that are options for you and what the possible side effects are. Your treatment depends on which organs are involved and how severe your condition is. Your doctor may prescribe:
- Corticosteroids to control inflammation. Medications such as prednisone help suppress the immune system and reduce inflammation of the blood vessels.
Other drugs that suppress your immune system. Several other drugs may help decrease the function of the immune system cells causing inflammation. They include cyclophosphamide, azathioprine (Azasan, Imuran) and methotrexate (Rheumatrex, Trexall).
Rituximab (Rituxan) is another option for treating granulomatosis with polyangiitis. It may be as effective as cyclophosphamide in treating severe cases, with fewer side effects.
Once the condition is under control, some drugs may be continued long term to prevent relapse. These include rituximab, methotrexate, azathioprine and mycophenolate (CellCept). A study including about 150 people who have granulomatosis with polyangiitis showed that those who discontinued their drugs or used lower doses had a higher relapse rate.
Side effect treatments
The drugs used to treat granulomatosis with polyangiitis have the potential to cause serious side effects, such as lowering your body's ability to fight infection. Your doctor will monitor your condition while you're taking these drugs and possibly prescribe drugs such as the following to help prevent side effects:
- Sulfamethoxazole-trimethoprim (Bactrim, Septra) to prevent some lung infections
- Bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use
- Folic acid, a synthetic form of the B vitamin folate, to prevent sores and other signs and symptoms associated with the depletion of folate in your body from methotrexate use
- Plasma exchange. Also known as plasmapheresis, this treatment removes the liquid portion of your blood (plasma) that contains disease-producing substances. Then you receive an infusion of 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.
- Kidney transplant. With advanced disease, you may need a kidney transplant to restore normal kidney function. Talk with your doctor to determine whether a kidney transplant might be an option for you. Your eligibility to receive a donated kidney will depend, in part, on how your other organs have been affected by the disease.
Coping and support
With treatment you're likely to recover from granulomatosis with polyangiitis. Even so you may feel stress about possible relapse or damage the disease may cause. Here are some suggestions for coping with the disease:
- Understand your condition. The more you know about granulomatosis with polyangiitis, the better prepared you may be to deal with complications, the side effects of your medications and relapses. Besides talking with your doctor, you may want to talk with a counselor or a medical social worker.
- Maintain a strong support system. Family and friends can help you cope. And you may 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 an appointment
You're likely to start by seeing your primary care doctor. He or she may refer you to a specialist in the lungs (pulmonologist), ear, nose, and throat (otolaryngologist), or kidneys (nephrologist). Unless your primary care doctor has some experience with granulomatosis with polyangiitis, you likely won't receive a diagnosis until you're seen by a specialist.
Here's some information to help you get ready for your appointment.
What you can do
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared.
- Be aware of any 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 any X-rays to the doctor you're about to see. Or pick up the material yourself to be sure it gets to your new doctor.
- List any symptoms you're experiencing, including those that may seem unrelated to the reason for which you scheduled the appointment.
- List key personal medical information, including other recent health problems, major stresses you've had, and all medications, vitamins and supplements you're taking.
- Consider taking a family member or friend with you to the appointment. Someone who accompanies you can help remember what the doctor says.
- Get a referral, if necessary. Some insurance companies require referrals for visits to specialists. If you're being referred to a specialist, be sure a letter of referral has been sent to the doctor, or bring it with you.
- List questions to ask your doctor.
For the signs and symptoms of granulomatosis with polyangiitis, some basic questions to ask your doctor include:
- What is likely causing my symptoms? What are other possible causes?
- What kinds of tests will I need? Do these tests require any special preparation?
- Is my condition temporary?
- What treatment do you recommend?
- What side effects can I expect from the treatment?
- 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 I need to see a specialist? Will I need a referral?
- Will I need to take medications? If so, is there a generic alternative to the medicine you're prescribing?
- Do you have any brochures or other printed materials that I can take with me? What websites do you recommend?
- Do you know of any support groups nearby?
What to expect from your doctor
Examples of questions your doctor may ask, include:
- When did you first begin experiencing symptoms?
- Do you smoke?
- Have your symptoms been continuous or occasional?
- Have you taken your temperature? If so, what was it?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- What, if anything, appears to worsen your symptoms?
What you can do in the meantime
If your symptoms are getting worse, tell your primary care doctor so that he or she can try to get you to a specialist quickly.