Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs.
Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.
Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.
Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal, most commonly from kidney failure.
Signs and symptoms of Wegener's granulomatosis may develop suddenly or over several months.
The first Wegener's granulomatosis symptoms usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys. Signs and symptoms may include:
For some people, the disease is limited to the lungs and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease, and may be detected only by blood and urine tests. However, in time kidney failure and anemia often occur.
When to see a doctor
The cause of Wegener's granulomatosis is unknown. It appears to develop after an initial inflammation-causing event triggers an abnormal reaction from your immune system. The combination of these events can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas). The triggering event may be an infection, but no specific infection has been identified as the cause.
Wegener's granulomatosis can begin at any age, but it most often starts in middle age, with an average onset between 40 and 65. Whites are more likely than blacks to develop Wegener's granulomatosis.
Besides affecting your upper and lower respiratory tracts, Wegener's granulomatosis may affect other organs, including your skin, eyes, ears, kidneys, spinal cord and heart. Complications may include:
Preparing for your appointment
You're likely to start by seeing your primary care doctor. However, you may be referred to a specialist, most commonly a lung (pulmonary) specialist, an ear, nose and throat (ENT) specialist, or a kidney specialist (nephrologist). Unless your primary care doctor has some experience with Wegener's granulomatosis, it's unlikely that a diagnosis will be made until you're seen by a specialist.
Because appointments can be brief, and there's often a lot of ground to cover, it's a good idea to arrive well prepared. Here's some information to help you get ready for your appointment, and what to expect from your doctor.
What you can do
Your time with your doctor is limited, so preparing a list of questions will help make the most of your time together. List your questions from most important to least important, in case time runs out. For the symptoms of Wegener's granulomatosis, some basic questions to ask include:
What to expect from your doctor
What you can do in the meantime
Tests and diagnosis
Besides asking you about your signs and symptoms, conducting a physical exam, and taking a medical history, your doctor may request several tests, including:
Treatments and drugs
With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. Maintenance therapy often needs to be continued for 18 to 24 months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition closely after treatment.
When standard treatments aren't effective, some doctors who are experienced in treating Wegener's use experimental drugs. Drugs currently under investigation include mycophenolate mofetil (CellCept), infliximab (Remicade), intravenous immunoglobulin, deoxyspergualin and antithymocyte globulin.
Side effect treatments
Coping and support
With treatment, you're likely to recover from Wegener's granulomatosis. However, you may feel stress about the possibility of recurrence or about any damage the disease may cause, such as to your kidneys. Here are some suggestions for coping with the disease:
Last Updated: 2012-12-19
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