Wegener's Granulomatosis

WHAT YOU SHOULD KNOW:

  • 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) may form inside the vessels. Granulomas may block the blood flow to other body organs and damage your organs and tissues. The exact cause of WG is unknown but a bacterial (germ) infection may be what starts the disease process. The inflammation and granulomas may lead to kidney failure, and could be life-threatening. WG may also cause lung problems making it hard for you to breathe. You may also have problems with your joints, skin, eyes, and heart.

  • You may need to have a biopsy, blood and urine tests, and imaging tests to check your condition. Medicines may be needed to treat your WG. You may also need to have your blood treated with plasma exchange. Surgery to fix other problems caused by WG may also be needed. If your kidneys fail, they may need to be removed. You may need a kidney transplant to replace your damaged kidneys with a donor (another person) one. 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.

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.

RISKS:

  • Medicines used to treat WG may cause you to have watery bowel movements. Medicines may also increase your risk for infection. Your muscles may shrink and you may gain weight. You may have a decreased desire for having sex. Females may have trouble getting pregnant. Males may become infertile (unable to get their partner pregnant.) Your risk for osteoporosis (brittle bones) and cataracts (clouding in the lens of your eyes) may increase. Your urinary bladder may swell and you may have lung, blood, kidney, and heart problems. Problems in your kidneys may lead to an increase in your blood pressure.

  • Treatment for WG may also cause diabetes (increased blood sugar level). Over time, you may get cancer in body areas such as your bladder, gallbladder, or breast. Surgery may cause infection, and blood clots may form in the deep veins of your body. Even with treatments, your signs and symptoms may come back, get worse, or cause death. If WG is left untreated, you may have a nasal blockage or discharge, and total hearing or vision loss. Your airway may narrow, and your kidneys may fail. These problems may lead to worsening of your condition, and you may die. Ask your caregiver if you have questions or concerns about your condition, treatment, or care.

WHILE YOU ARE HERE:

Informed consent

is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

An IV

is a small tube placed in your vein that is used to give you medicine or liquids.

Vital signs:

Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.

Medicines:

You may be given the following medicines:

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

  • Antiemetic medicine: Antiemetic medicine may be given to calm your stomach and help control vomiting (throwing up).

  • 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.

Tests:

  • 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 infection. He may also test your blood to check your level of WG antibodies. 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.

Treatment options:

  • 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 caused damage to your kidney. 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.

    • Tracheostomy: A tracheostomy is a tube put into your trachea (windpipe) to help you breathe. A small opening is made in your neck called a stoma. This may be done if you have severe trouble breathing.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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.

Learn more about Wegener's Granulomatosis (Inpatient Care)

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