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Granulomatosis with polyangiitis (GPA) is a rare disease that causes swelling, also called inflammation, of small blood vessels. This condition mainly affects blood vessels in the nose, sinuses, throat, lungs and kidneys. But it can affect any organ.
GPA used to be called Wegener granulomatosis. It's one of a group of blood vessel diseases called vasculitis. GPA slows blood flow to some organs. The affected tissues can develop inflamed areas called granulomas. Granulomas can damage these organs and affect how they work.
Symptoms of GPA vary. At first, the symptoms can seem like those of a cold. Other symptoms depend on which organs the disease affects. The cause of GPA isn't clear, but the immune system may play a role.
It's important for healthcare professionals to find and treat GPA as soon as possible. Early treatment can help people live full lives. Without treatment, the condition can lead to organ damage that sometimes is fatal.
Symptoms of granulomatosis with polyangiitis (GPA) can vary widely from person to person. Symptoms can start quickly, or they can develop over months.
Early on, GPA can cause general symptoms such as:
Specific symptoms depend on what parts of the body GPA affects.
If GPA affects the ears, nose or throat, symptoms can include:
If GPA affects the lungs or windpipe, symptoms can include:
For some people, the disease affects only the lungs. Without treatment, GPA can lead to lung bleeding and scarring over time.
If GPA affects the kidneys, you might not have symptoms. Or you might have symptoms such as:
If GPA affects the eyes, symptoms can include:
Other symptoms of GPA can include:
GPA symptoms can seem like those of some other health conditions. Early symptoms may feel like a cold or flu and can last for weeks or longer. See your healthcare professional if you have a runny nose that doesn't get better with cold medicines. Be sure to get a healthcare checkup if you have a runny nose along with:
The disease can become worse quickly. Without treatment, it can lead to other serious health conditions called complications. That's why it's important for a healthcare professional to find it and start treatment early.
The exact cause of granulomatosis with polyangiitis (GPA) isn't clear. The immune system may play a role. Some white blood cells may not function as they should, and they may be involved in the disease. The immune system makes proteins called antibodies that protect the body from germs such as viruses and bacteria. GPA has been linked with the presence of certain antibodies that attack healthy cells by mistake.
The condition can lead to inflamed, narrowed blood vessels and harmful, inflamed tissue masses called granulomas. Granulomas can destroy healthy tissue. And narrowed blood vessels lower the amount of blood and oxygen that reaches tissues and organs.
GPA doesn't spread from person to person. And it likely doesn't pass from parents to children through genes.
Age is a risk factor for granulomatosis with polyangiitis. Most often, the disease affects people between the ages of 40 and 65. But it can happen at any age.
Granulomatosis with polyangiitis (GPA) can lead to other health conditions called complications. The complications depend on which organs or body parts the disease affects. They can include:
Diagnosis involves the steps that your healthcare team takes to find out if you have granulomatosis with polyangiitis (GPA). Your healthcare professional asks you about your symptoms and health history. You also get a physical exam. You may need other tests as well.
Blood tests can check for:
Urine tests can reveal if the urine has red blood cells or too much protein. These might mean that the disease is affecting the kidneys.
If you have lung symptoms, chest X-rays and CT scans can help find out if granulomatosis with polyangiitis is the cause. CT scans use X-rays and a computer to make more-detailed images of the organs inside the body. CT scans also can help find out if granulomatosis with polyangiitis is the cause of head or neck symptoms. Once a person starts treatment for GPA, healthcare professionals can use CT scans to find out if treatment is working.
During a biopsy, a healthcare professional removes a small sample of tissue from the affected area of the body. For example, a tissue sample could be taken from the lungs, skin, kidneys or inside of the nose. The tissue sample is checked with a microscope to look for signs of inflammation or damage. This test can confirm if you have granulomatosis with polyangiitis.
Treatment for granulomatosis with polyangiitis (GPA) includes medicines to get the disease under control and keep it from coming back. Along with getting care from your regular healthcare professional, you'll likely need treatment and follow-up care from various specialists. The specialists that you need depend on the organs that the disease affects. With early treatment, many people who have GPA get better and lead full lives.
Medicines called corticosteroids help lessen the immune system response and lower inflammation of the blood vessels. Common side effects include weight gain, risk of infection and thinning of bones.
Other medicines that lessen the immune system response include:
Once your condition is under control, you might stay on some medicines long-term. You might hear your healthcare professional call this maintenance therapy. The goal with long-term treatment is to prevent GPA from coming back, also called relapse. Medicines that can be used long-term include rituximab, methotrexate, azathioprine and mycophenolate.
Medicines such as these can raise the risk of infection. Cyclophosphamide can cause upset stomach, diarrhea and hair loss. Your healthcare professional may prescribe other medicines to help prevent side effects from prescribed treatments.
Plasma exchange removes the liquid portion of blood, called plasma. In most people with GPA, plasma has proteins in it that are linked with the disease. During plasma exchange, you receive fresh plasma or a protein made by the liver called albumin. Albumin helps the body make new plasma. Sometimes, plasma exchange is used to treat people who have very serious GPA. It can help the kidneys get better. Another name for this treatment is plasmapheresis.
You're likely to get better after treatment for granulomatosis with polyangiitis (GPA). Even so, you might feel stress about the disease coming back or the damage it can cause. Here are some tips to help you cope:
You're likely to start by seeing your usual healthcare professional. You also might be referred to a specialist such as:
A specialist likely will be the one to find out if you have granulomatosis with polyangiitis.
Here's some information to help you get ready for your appointment.
Be aware of any rules to follow before your healthcare checkup. When you make the appointment, ask if you need to do anything in advance. For instance, you may be told to limit your diet.
If you've had any recent blood tests or chest X-rays at another medical office or hospital, ask the staff there to forward the test results and X-rays to the healthcare professional you're seeing. Or pick up the material yourself to be sure it gets to your healthcare professional.
Get a referral if your insurance company requires it for visits to specialists. Be sure a letter of referral has been sent to the healthcare professional or bring the letter with you.
Before your appointment, also make a list of:
Take a family member or friend with you to the appointment if you can. This person can help you remember the information you receive.
For granulomatosis with polyangiitis, questions to ask your healthcare team include:
Your healthcare professional is likely to ask questions such as:
If your symptoms are getting worse, tell your main healthcare professional. That way, your healthcare professional can try to get you to a specialist quickly.