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Granulomatosis with polyangiitis
WHAT YOU SHOULD KNOW:
- Granulomatosis with polyangiitis (GPA) 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 GPA 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. GPA 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 GPA. You may also need to have your blood treated with plasma exchange. Surgery to fix other problems caused by GPA 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.
Take your medicine as directed.
Call your healthcare provider if you think your medicine is not helping or if you have side effects. Tell him if you are allergic to any medicine. Keep a list of the medicines, vitamins, and herbs you take. Include the amounts, and when and why you take them. Bring the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency.
- Antibiotics: This medicine is given to fight or prevent infection caused by bacteria. This may also help prevent GPA from coming back. Stopping antibiotics without your caregiver's OK may make the medicine unable to kill all of the germs. Never save antibiotics or take leftover antibiotics that were given to you for another illness.
- 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.
- Ask your caregiver when to return for a follow-up visit. Tell your caregiver about any new signs and symptoms you have. Tell your caregiver if you are having side effects from your medicines. The kind and amount of medicine you are taking may need to be changed. The goal is for the medicines to work well, while not causing you to have bad side effects. You and your caregiver will talk about how long you may need to take each of your medicines.
- You may need repeat chest x-rays and CT scans to check your lungs. Blood and urine tests may also be done to check how much medicine is in your blood, or how well it is working. You may need to have these tests more than once. Keep all appointments. Write down any questions you may have. This way you will remember to ask these questions during your next visit.
You may need to eat foods high in folic acid or take a folic acid supplement. Immunosuppressive medicines often decrease the amount of folic acid in your body. Ask your caregiver for information on foods high in folic acid and about taking supplements.
Men 19 years old and older should drink about 3 liters of liquid each day (close to 13 eight-ounce cups). Women 19 years old and older should drink about 2.2 liters of liquid each day (close to 9 eight-ounce cups). Good choices for most people to drink include water, juice, and milk. If you are taking immunosuppressive medicine, you need to make sure you drink enough fluid. Ask your caregiver how much liquid you should drink each day.
Use lotion and petroleum jelly on your nose if it is dry and crusted. Nasal irrigation may also decrease dryness and crusting in your nose. These will help soothe and soften your dry nasal passages. Ask your caregiver for more information about these options.
It is never too late to quit smoking. Smoking harms your heart, lungs, and blood. You are more likely to have a heart attack, lung disease, and cancer if you smoke. You will help yourself and those around you by not smoking. Your risk of having bad effects from immunosuppressant medicine may increase if you smoke. Ask your caregiver for more information about how to stop smoking if you are having trouble quitting.
CONTACT A CAREGIVER IF:
- You have a fever (high body temperature).
- 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.
SEEK CARE IMMEDIATELY IF:
- You had surgery and pus is draining from your wound.
- 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.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.