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.
Symptoms & causes
Dec. 22, 2015 Print Share on: Facebook Twitter
Causes of GPA and MPA
Preventing a GPA or MPA relapse
Diet and MPA or GPA
Does GPA or MPA run in families?
GPA and MPA treatment options
Living with GPA or MPA
Signs of GPA
Kidney donation: Are there long-term risks?
Sed rate (erythrocyte sedimentation rate)
Blood in urine (hematuria)
Shortness of breath
Coughing up blood
Unexplained weight loss
Granulomatosis with polyangiitis
Symptoms & causes
Diagnosis & treatment
Preparing for your appointment
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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:
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.
By Mayo Clinic Staff Print
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.