Wegener's granulomatosis often begins with inflammation of small and medium-sized blood vessels and tissues in the nose, sinuses, throat, or lungs. It may progress to inflammation of blood vessels throughout the body (generalized vasculitis).

• The cause is unknown.
• The disorder usually begins with nosebleeds, nasal congestion with crusting, sinusitis, hoarseness, 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 usually another drug that suppresses the immune system are needed to control inflammation.

Wegener's granulomatosis can occur at any age. 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. Various organs in the body are damaged and malfunction because the arteries that supply them with blood are damaged. Wegener's granulomatosis 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:

• Nosebleeds, sometimes severe
• Nasal congestion with crusting in and around the nose
• Collapse of the bridge of the nose, causing it to sag
• A hole in the nasal cartilage that separates one side of the nose from the other (nasal septum)
• Sinusitis
• Hoarseness
• Middle ear infections (otitis media)
• Difficulty breathing
• Coughing (sometimes with blood)

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. Difficulty breathing may result from bleeding in the lungs, which requires immediate medical attention.
• Joints: Joints may become swollen and ache.
• Nerves: A limb may feel numb, tingly, or weak, or vision may be impaired. People may see double and, without treatment, may become blind.
• 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.

Anemia is common and can be severe.

Diagnosis

Wegener's granulomatosis must be diagnosed and treated early to prevent complications, including kidney disorders, lung disorders, and heart attacks.

Doctors usually suspect the diagnosis based on the distinctive pattern of symptoms. 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. For example, a chest x-ray may show cavities or dense areas in the lungs that look like cancer.

Although blood test results cannot specifically identify Wegener's granulomatosis, they can strongly support the diagnosis. One such test can detect antineutrophil cytoplasmic antibodies in the blood. These antibodies attack certain white blood cells and occur in several types of vasculitis. Urine tests also help support the diagnosis. People with this disorder may have blood or too much protein in their urine.

Doctors can confirm the diagnosis only 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 also be helpful.

Treatment

With treatment, symptoms usually disappear (called remission). However, in about half of treated people, symptoms return (called a relapse). Relapses may occur when treatment is stopped or many years later.

Corticosteroids are almost always used to suppress inflammation. Most people also need drugs that suppress the immune system (immunosuppressants), such as cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
(usually used when the disorder is severe), methotrexateSome Trade Names
TREXALL
, or azathioprineSome Trade Names
IMURAN
. Most people feel better within days to weeks. But for some, improvement may take months.

During remission, the dose of the drugs is reduced. Treatment is usually continued for at least a year 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 recur, 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. CyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
can cause bladder irritation and sometimes bladder cancer. A complete blood count is done frequently, sometimes as often as once a week for patients who take strong immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease.

People with Wegener's granulomatosis 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.

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.

Last full review/revision April 2008 by Carmen E. Gota, MD