Wegener's Granulomatosis

What is Wegener's granulomatosis?

Wegener's granulomatosis (WG) is a disease where your small and medium-sized blood vessels are inflamed (swollen). The cells lining these vessels may die, and granulomas (small lumps) often form. Granulomas may block the blood flow to other body organs and damage your organs and tissues. The inflammation and granulomas may lead to kidney failure, and could be life-threatening. With WG, your lungs may not function properly. You may have problems with your joints, skin, eyes, and heart. Having your condition treated may decrease your symptoms such as trouble breathing. Treatment may also slow the disease and prevent further damage to your kidneys and other body organs.

What may cause Wegener's granulomatosis?

The exact cause of WG is not known. Caregivers believe a bacterial (germ) infection may start the disease process. This infection may cause damage to your immune system, and it may not work as it should. The immune system is your body's defense against infection and disease. Antibodies are special substances in your immune system that fight germs and protect your body. When your immune system is damaged, your immune cells may make antibodies that attack your own body's cells. Ask your caregiver for more information about possible causes of WG.

What are the signs and symptoms of Wegener's granulomatosis?

With WG, you may tire easily, have muscle and joint pain, and a fever (high body temperature). You may also have any of the following depending on the organs and tissues damaged by WG:

  • Lung and breathing problems: You may have a high-pitched noise when you breathe and a cough. Inflammation in your lungs may also make it hard for you to breathe.

  • Kidney and urination problems: You may have blood in your urine. You may also urinate less than you usually do.

  • Eye and vision problems: You may have bulging, swollen, red, or painful eyes. You may also have watery eyes and blurry vision. With WG, you may be at risk of losing your eyesight.

  • Ear and hearing problems: You may have ear infections often. You may also have trouble hearing or have complete hearing loss.

  • Nasal (nose) problems: You may have a stuffy, dry nose with crusting in and around your nose. You may also have a runny nose, and you may have nose bleeds. You may have a saddle nose defect which is damage to your nose caused by your WG. You may also have trouble smelling.

  • Mouth and throat problems: You may have sores in your mouth and trouble swallowing. Your gums and parotid glands may become red and swollen. The parotid glands are what make your saliva (spit).

How is Wegener's granulomatosis diagnosed?

A complete physical exam may be done by your caregiver. He will ask about any signs and symptoms you are having. Tell your caregiver about any medical conditions you have, or have had in the past. You may need any of the following:

  • Biopsy: During a biopsy, your caregiver will take a tissue sample from your body and send it to a lab for tests. The tissue sample is often taken from a body organ that may be damaged by WG. The biopsy area will be numbed with medicine before the tissue is removed. Your caregiver may need to make an incision (cut) in your skin to get a tissue sample. After the biopsy, you may have stitches and a bandage to close and cover your biopsy site.

  • Blood tests: You may need blood taken for tests. The blood can be taken from a blood vessel in your hand, arm, or the bend in your elbow. It is tested to see how your body is doing. Your caregiver may test your blood to look for an infection. He may also test your blood to check for the antibodies that cause WG. You may need to have blood drawn more than once.

  • Bronchoalveolar lavage: Your caregiver will use a bronchoscope (a long tube with light on the end) to do this test. The scope will be put into your mouth and down into your lungs. Your caregiver may give you pain medicine or medicine to help you relax for the test. Special liquid will be put in through the scope and into your lungs. Your caregiver will then suction the liquid out and send it to a lab for tests.

  • Imaging tests:

    • Chest x-ray: A chest x-ray is a picture of your lungs and heart. The x-ray may help your caregiver learn if your WG has damaged the blood vessels in these organs. Extra fluid, nodules (small masses), and thickening of your airway walls may also be seen. Your caregiver can also check if your heart is enlarged.

    • Computed tomography scan: This test is also called a CT scan. A special x-ray machine uses a computer to take pictures of your sinuses, and the area around your eyes. Sinuses are hollow air spaces found within your skull bones. Your caregiver may also scan your chest. Before taking the pictures, you may be given dye through an intravenous (IV) tube in your vein. The dye helps the pictures show up better. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to shellfish or have other allergies.

    • Magnetic resonance imaging: This test is also called an MRI. During the MRI, pictures may be taken of your head and other areas of your body. Your caregiver will look for swelling, granulomas, and damage to your facial bones. He will also check for damage to other areas of your body. You will need to lie still during an MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. This can cause serious injury.

  • Urine sample: A sample of your urine is collected and sent to a lab for tests.

How is Wegener's granulomatosis treated?

The goal of treatment is to decrease your signs and symptoms. Your caregiver will work with you to improve your condition and help you feel better. Ask your caregiver for more information about the following treatments:

  • Medicine:

    • Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.

    • Immune globulins: This medicine is given as a shot or an IV infusion to make your immune system stronger. You may need immune globulins to treat or prevent an infection. It is also used when you have a chronic condition, such as lupus or arthritis. You may need many weeks of treatment. Each infusion can take from 2 to 5 hours.

    • Immunosuppressive therapy: This medicine may be given to slow down your immune system. Your immune system may be seeing your normal cells as abnormal. This medicine may help prevent your immune system from harming your body.

    • Steroids: Steroid medicine may be given to decrease inflammation which is redness, pain, and swelling. Steroids may be given together with your immunosuppressive medicine. There are many different reasons to take steroids. This medicine can help a lot but may also have side effects. Be sure you understand why you need steroids. Do not stop taking these medicines without your caregiver's OK. Stopping on your own can cause a bad response.

  • Plasma exchange: Plasma exchange is done if you have life-threatening signs and symptoms, such as trouble breathing. An IV tube is put into your vein and some of your blood is removed. A special machine spins and separates the plasma (fluid portion) from the blood cells. The plasma from your blood is then removed and replaced with plasma from a donor (another person). The blood cells and the replacement plasma are then put back into your body through the IV. Ask your caregiver for more information about plasma exchange as a treatment option.

  • Surgery: Surgery may be done to slow the progress of your disease. It may also be done to help prevent your signs and symptoms from getting worse. Surgery may also be needed if your health does not get better after being treated with immunosuppressant medicine. Ask your caregiver for more information about the following:

    • Endoscopic sinus surgery: This surgery is done to remove nasal crust and control nose bleeding. This may be done using an endoscope (a long tube with a lens and light on the end).

    • Saddle nose deformity surgery: Saddle nose deformity is commonly caused by scarring and erosion (wearing away) of the bones in your nose. You may need surgery to fix your nose and decrease symptoms caused by the deformity.

    • Septal perforation surgery: This surgery is done to fix large holes in your nasal septum (tissue separating your nostrils).

    • Subglottic stenosis surgery: Subglottic stenosis is a condition where the muscles in your airway, below your larynx (voice box), have narrowed. You may need surgery to widen the narrowed areas and help you breathe easier.

    • Kidney transplant: You may need this surgery if WG causes your kidneys to fail. During surgery, your damaged kidney is removed and replaced with a healthy kidney. The matched donor kidney may come from a relative or another person. Ask your caregiver for more information about this option.

Where can I find support and more information?

Wegener's granulomatosis is a life-changing disease. You and those close to you may be scared, sad, and angry. These feelings are normal. Talk to your caregiver, your family, and your friends about your feelings. You may want to join a support group. A support group is a group of people who also have WG. Learning as much as you can about WG may help you cope with your illness better. Contact any of the following:

  • National Kidney Foundation
    30 East 33rd Street
    New York , NY 10016
    Phone: 1- 212 - 889-2210
    Phone: 1- 800 - 622-9010
    Web Address: http://www.kidney.org
  • Vasculitis Foundation
    P.O. Box 28660
    Kansas City , MO 64188
    Web Address: www.vasculitisfoundation.org/

When should I call my caregiver?

Call your caregiver if:

  • You have a fever.

  • You have blurry eyesight.

  • You are urinating less than usual.

  • You have red and swollen eyes.

  • You have questions or concerns about your condition or treatment.

When should I seek immediate help?

Seek care immediately or call 911 if:

  • You have blood in your urine.

  • You have new hearing loss.

  • You have new loss of vision.

  • You have shortness of breath or trouble breathing all of a sudden.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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