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Granulomatosis with Polyangiitis

(Previously known as Wegener's Granulomatosis)

By

Alexandra Villa-Forte

, MD, MPH, Cleveland Clinic

Last full review/revision Jun 2019| Content last modified Jun 2019
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NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
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Granulomatosis with polyangiitis often begins with inflammation of small- and medium-sized blood vessels and tissues in the nose, sinuses, throat, lungs, or kidneys.

  • The cause is unknown.

  • The disorder usually begins with nosebleeds, nasal congestion with crusting, sinusitis, hoarseness, ear pain, fluid in the middle ear, eye redness and pain, wheezing, and coughing.

  • Other organs may be affected, sometimes with serious complications, such as kidney failure.

  • Symptoms and other findings suggest the diagnosis, but a biopsy is usually needed to confirm it.

  • A corticosteroid and a second drug that suppresses the immune system are needed to control inflammation and induce remission.

Granulomatosis with polyangiitis is most common among whites but can occur in all ethnic groups and at any age. Most people are affected at about age 40. Its cause is unknown. It resembles an infection, but no infecting organism has been identified. Collections of immune cells that cause inflammation (called granulomas) form nodules and ultimately destroy normal tissue. Granulomatosis with polyangiitis is often life threatening.

Symptoms

The disorder may begin suddenly or gradually. Usually, the first symptoms involve the upper respiratory tract—the nose, sinuses, ears, and windpipe (trachea). They may include the following:

  • Nasal congestion with crusting in and around the nose, sometimes with bloody discharge

  • Collapse of the bridge of the nose, causing it to sag (saddle nose deformity)

  • A hole in the nasal cartilage that separates one side of the nose from the other (nasal septum)

  • Sinusitis and sinus pain

  • Hoarseness

  • Inflammation in the middle ear (otitis media), typically with ear pain and sometimes loss of hearing

  • Difficulty breathing

  • Coughing (sometimes with blood)

  • Pain in the voice box (larynx), wheezing, and rarely a loud squeaking noise when breathing in (stridor)

Sometimes only the upper respiratory tract is affected for many years. People may also have a fever, feel generally unwell, and lose their appetite. Inflammation can affect the eyes, which may become swollen, red, and painful.

The disorder may progress to affect other areas of the body or may affect several organs from the beginning:

  • Lungs: The lungs are usually affected at some point. People may feel short of breath and cough, sometimes cough up blood. Difficulty breathing may result from bleeding in the lungs, which requires immediate medical attention.

  • Joints: Joints and muscles often ache. Arthritis may develop.

  • Nerves: A limb may feel numb, tingly, or weak, or vision may be impaired. Nerves may be damaged. People may see double and, without treatment, may become blind. The eyes may bulge and be painful because inflammation causes tissues to build up and press on other structures in the orbit.

  • Skin: A rash or sores may appear on the skin.

  • Kidneys: The kidneys are often affected. Kidney function may be slightly or severely impaired. Severe kidney damage causes high blood pressure and swelling due to fluid retention (edema). Life-threatening kidney failure may occur. However, kidney damage can proceed without causing symptoms.

  • Veins: Blood clots (deep vein thrombosis) can develop in the legs.

Anemia is common and can be severe.

Diagnosis

  • A doctor's evaluation

  • Imaging tests

  • Blood and urine tests

  • Biopsy

Granulomatosis with polyangiitis must be diagnosed and treated early to prevent complications, including kidney disorders, lung disorders, and heart attacks.

Doctors usually suspect the diagnosis of granulomatosis with polyangiitis based on the distinctive pattern of symptoms. For example, doctors suspect the diagnosis when people have unexplained respiratory symptoms (including otitis media in adults, which is otherwise unusual) particularly if problems in other organs, especially the kidneys, exist. Doctors also suspect the diagnosis in people who have a long history of sinus problems that have not been cured by or only partially cured by antibiotics.

A chest x-ray is done because the lungs are usually affected. However, symptoms and chest x-rays can resemble those of several lung disorders, making the diagnosis difficult. So doctors also do a computed tomography (CT) scan of the chest to look for things the chest x-ray might miss, such as cavities or areas of inflammation in the lungs that may mimic cancer or infection.

Although blood test results cannot specifically identify granulomatosis with polyangiitis, they can strongly support the diagnosis. One such test can detect antineutrophil cytoplasmic antibodies (c-ANCA) in the blood. These antibodies occur in several types of vasculitis and trigger certain white blood cells to attack normal organs. Other specific antibodies are identified with further testing. People with this disorder may have blood and protein in their urine due to kidney inflammation. Because kidney inflammation can cause damage without causing symptoms, doctors always do a urine test to detect this inflammation.

Doctors can confirm the diagnosis by examining a small piece of tissue under a microscope (biopsy). The tissue sample may be taken from an affected area, such as the nasal passages, airways, or lungs. Skin and kidney biopsies may occasionally be helpful.

Prognosis

The prognosis depends on how severe and widespread the disorder is, how much organ damage has occurred, and how quickly people receive treatment.

For people with severe disease, prognosis has been greatly improved by using drugs that suppress the immune system (immunosuppressants). With treatment, symptoms completely disappear (called remission) for about 80% of people. However, in about half of treated people, symptoms return (called a relapse). Relapses may occur when treatment is stopped or many years later. Resuming or increasing treatment can usually control the disorder.

Treatment

  • Corticosteroids

  • Other immunosuppressants

Corticosteroids (such as prednisone) are almost always used to suppress inflammation. Most people also need immunosuppressants, such as cyclophosphamide, methotrexate, rituximab, or azathioprine. If the disorder is not severe, doctors give a corticosteroid and methotrexate. Rituximab may be given instead of methotrexate. If the disorder is severe, people are hospitalized and are given high doses of a corticosteroid and cyclophosphamide or rituximab. Most people feel better within days to weeks. But for some, improvement may take months.

During remission, the dose of the drugs is gradually reduced. Treatment of granulomatosis with polyangiitis is usually continued for at least a year or often for many years after the symptoms disappear. The dose of a corticosteroid can usually be gradually decreased and eventually stopped. Doses may need to be adjusted throughout the course of treatment. If symptoms worsen or return, the dose is increased, or if drugs have been stopped, they are started again.

Because immunosuppressants weaken the immune system, the risk of developing serious infections is increased. Taking prednisone for a long time can result in weight gain, cataracts, high blood pressure, decreased bone density, diabetes, changes in mood, and difficulty sleeping. Cyclophosphamide can cause bladder irritation and, if taken repeatedly, sometimes bladder cancer. When cyclophosphamide is given by vein (intravenously), mesna, which chemically counteracts some toxic effects of cyclophosphamide on the bladder, is also given.

People with granulomatosis with polyangiitis need to be closely monitored by their doctor to check whether the dose of the drugs is appropriate, whether they are having side effects from the drugs, whether they could have an infection, and, during remission, whether there is any indication of a relapse. A complete blood count is done frequently, sometimes as often as once a week, for people who take potent immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease.

People should also learn as much as they can about the disorder. Thus, they can recognize signs of a relapse early. People can also learn to test their urine for blood and protein so that they can notify their doctor at the first sign of any new abnormality.

If people develop chronic kidney disease with kidney failure, they may need a kidney transplant.

More Information

Drugs Mentioned In This Article

Generic Name Select Brand Names
CYTOXAN (LYOPHILIZED)
IMURAN
OTREXUP
RAYOS
RITUXAN
MESNEX
NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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