Wegener’s granulomatosis, now known as granulomatosis with polyangiitis (GPA), is a disease that causes swelling and irritation of blood vessels in the kidneys, nose, sinuses, throat and lungs. Swollen blood vessels make it harder for blood to get to the organs and tissues that need it, which can be harmful. The disease also causes lumps called granulomas to form and damage the area around them. In some people GPA only affects the lungs. GPA that affects the kidneys can lead to chronic kidney disease and kidney failure.

The first signs of GPA are usually in your nose, sinuses, throat or lungs. You may notice a runny nose, sinus infections or nosebleeds. Other signs might include:

  • Coughing 
  • Trouble catching your breath
  • Wheezing
  • Fever 
  • Feeling achy 
  • Numbness in your arms, legs, fingers or toes

You might not notice any symptoms if GPA is affecting your kidneys. Signs of kidney problems might only show up in blood and urine tests, but if you do have symptoms, you might notice blood in your urine. GPA can get worse very quickly. If you notice a runny nose that won’t go away, bloody noses, or coughing up blood, see a doctor right away. GPA can get worse quickly and it is important to treat it early to prevent permanent organ damage, such as kidney failure.

If your doctor thinks you could have GPA, you might need to have blood and urine tests , chest X-rays, CT scans or MRI scans, and biopsies. If your tests show that you have GPA, your doctor might tell you to take medicines that control inflammation (swelling and irritation) and weaken your immune system. These medicines are called immunosuppressants. Another treatment option to weaken your immune system is called plasmapheresis (plaz-muh-fuh-REE-sis). Other treatments can help manage side effects and prevent complications. If GPA causes your kidneys to fail, you will need dialysis or a kidney transplant.

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