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Granulomatosis with polyangiitis

What is Granulomatosis with polyangiitis?

Harvard Health Publishing

Granulomatosis with polyangiitis (GPA) is a rare disease marked by inflammation of the blood vessels. The condition is potentially life-threatening.  It was formerly called Wegener's granulomatosis.

In GPA, inflammation damages the walls of small- and medium-sized arteries and veins. This damage interferes with normal blood supply to nearby tissues. As a result, tissues in many parts of the body may be injured and destroyed.

Tissues affected by GPA contain islands of inflamed cells. These are called granulomas.

Three areas in the body are affected most commonly:

In some cases, GPA involves other organs. This may include the eyes, ears, skin, joints, heart and nerves.

GPA is believed to be an autoimmune disorder. This means the body's immune defenses mistakenly attack the body's own cells and tissues. In this case, the misdirected immune attack is aimed at the blood vessels. This triggers inflammation and damage.


When GPA first develops, most people have upper respiratory complaints.  Common symptoms of GPA include:

As the disease gets worse, upper respiratory symptoms usually worsen. Other parts of the body can be affected, too.

The following symptoms may occur, depending on the location and severity of the inflammation:


Your doctor may suspect GPA if you have:

And your symptoms have worsened despite treatment for more common conditions, such as pneumonia or a sinus infection.

Many early symptoms of GPA are similar to those of milder and more common respiratory problems. As a result, the average person with GPA often has symptoms for months before the correct diagnosis is made.

Your doctor will review your symptoms and medical history. This will include any treatments that you have received so far.

Your doctor will conduct a thorough physical examination. He or she will carefully check your entire respiratory tract, from the tip of your nose to the base of your lungs. The doctor will look for evidence of inflammation and tissue injury. Your doctor may also examine your eyes, ears, heart and skin.

After the physical exam, your doctor may send you for tests. These tests will look for inflammation and organ damage. The tests will focus on your upper respiratory tract, lungs and kidneys. These tests may include:

These tests also help to check for other illnesses that may cause symptoms similar to GPA.

The only way to confirm this diagnosis is to have a biopsy. Tissue is taken from an affected organ. It is then examined under a microscope to check for granulomas and areas of inflammation.

Expected Duration

Granulomatosis with polyangiitis can rapidly lead to death in people with multiple affected organs that do not get appropriate treatment.

With treatment, symptoms can go away for long periods. However, setbacks are common. Most people are in treatment and under a doctor's supervision for at least two years from the time of diagnosis.


There is no known way to prevent granulomatosis with polyangiitis.


Initial treatment for granulomatosis with polyangiitis usually includes the anti-inflammatory corticosteroid drug prednisone.  For milder or more limited disease, your doctor may also recommend treatment with the drug methotrexate (Folex, Rheumatrex).

For active or severe disease, your doctor may recommend treatment with a combination of two drugs:  prednisone along with cyclophosphamide (Cytoxan, Neosar) or rituximab (Rituxan). Cyclophosphamide and rituximab are powerful immune-suppressing medications.

As your symptoms subside, your doctor will try to reduce the dosage of each drug gradually.

Once your condition improves and you've taken cyclophosphamide or rituximab for three to six months, your doctor may suggest switching medications. He or she may recommend methotrexate or azathioprine (Imuran, Azasan) for a total of one year of treatment. However, longer courses of therapy may be necessary if disease control is interrupted by periods of active disease.

Cyclophosphamide can cause serious side effects such as bladder damage (especially bleeding) and an increased risk of bladder cancer.  Cyclophosphamide and rituximab increase a person's risk of serious infections.

If you develop serious side effects related to these medications, your doctor may prescribe an alternative medication.

A procedure called plasmapheresis may help some people. In this procedure, blood is filtered and returned to the body. This procedure may help because it removes harmful antibodies but details about why it may work are unknown. 

Treatment options

The following list of medications are related to or used in the treatment of this condition.

View more treatment options

When To Call a Professional

See your doctor if you have a chronic stuffy nose, runny nose, nosebleeds or cough. Keep in mind that even with these symptoms, it's likely that you have a sinus or viral infection as these conditions are much more common than granulomatosis with polyangiitis.


With treatment, more than 85% of people with granulomatosis with polyangiitis survive for seven years or longer after diagnosis. With treatment, most people with the condition enter remission. However, as many as half will relapse when medication is reduced or stopped. Those who do not relapse may remain in remission for many years, often without needing more medication.

Additional Info

National Institute of Allergy and Infectious Diseases (NIAID)

American College of Rheumatology

Vasculitis Foundation

Learn more about Granulomatosis with polyangiitis

Treatment options

Care guides

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.