Granulomatosis with polyangiitis (GPA)

What is granulomatosis with polyangiitis (GPA)? 

GPA is a form of ANCA-associated vasculitis that can cause inflammation of small-to medium-sized blood vessels. GPA is a type of vasculitis that can affect your kidneys. In addition to inflammation, GPA also presents with granulomas, which are groups of white blood cells that build up because of inflammation throughout the body.  

Inflammation from GPA can affect your:  

• Kidneys 
• Lungs and trachea (the tube that connects your lungs to your nose and mouth)  
• Nose  
• Sinuses (the air-filled spaces behind your cheeks and forehead that open into your nose) 
• Eyes 

GPA is rare. It affects about 3 out of every 100,000 people in the United States. It is a serious disease that can get worse quickly. It can cause kidney failure, lung failure or death if it is not diagnosed and treated.  

GPA used to be called Wegener's granulomatosis, but that term is not used any longer.

What causes GPA?

Researchers are still looking into the causes of GPA, such as genetics, environmental factors, or infections. GPA is associated with anti-neutrophil cytoplasmic auto-antibodies (ANCA). Antibodies are proteins in your blood that identify and defend your body from invaders, like germs and viruses. ANCA are antibodies that mistake infection-fighting white blood cells for invaders and attack them. These attacks cause the release of destructive chemicals from the white blood cells, which leads to inflammation and damage of blood vessels.  

Similar to MPA, GPA affects small- to medium-sized  blood vessels throughout your body. This damage can lead to issues with different tissues and organs, such as the kidneys, lungs, eyes, ears and sinuses. GPA can affect people of any sex and at any age. It most often affects people:  

• Between the ages of 45 and 65  
• Whose ancestors came from Europe

What are the symptoms of GPA?

Sometimes the first symptoms of GPA may seem like a cold or the flu: 

• A runny nose that will not stop 
• Sores or crusty buildup in your nose 
• Chronic or repeated sinus infections 
• Nosebleeds  
• A cough, that may include coughing up blood 
• Trouble catching your breath or wheezing 

Other symptoms may include: 

• Pain in your:  
  • Nose or the area around your nose and eyes 
  • Ears  
  • Joints or muscles 
  • Chest 
• Feeling weak and tired 
• Fever  
• Feeling achy  
• Numbness in your arms, legs, fingers or toes 
• Sudden loss of vision or hearing 
• Feeling less hungry than normal, or weight loss 
• Rashes or sores on your skin 

GPA symptoms can happen suddenly or may develop over many months. Symptoms may get worse quickly. See a doctor if you have a runny nose or sinus infections that don't go away, especially if you also have nosebleeds, cough up blood, or have other symptoms of GPA.

How does GPA affect the kidneys? 

GPA inflames and damages the glomeruli, which are the filters in the kidneys. They are made up of tiny blood vessels called capillaries that clean your blood. The damage caused by GPA may lead to kidney failure. If your kidneys fail and do not recover, you will need dialysis or a kidney transplant. 

How do doctors treat GPA?

Doctors use different types of medicines to treat GPA. 

These act on inflammation, and may include:  

• B-cell-depleting medicines, which work by disturbing and changing B-cells, a type of white blood cell that produces antibodies
• Alkylating agents, which are medicines that have a harmful effect on immune cells
• Glucocorticoids, also called steroids, which suppress many parts of the immune system
• Complement system modulators, which are medicines that affect a part of the immune system that protects against bacteria and viruses 

GPA is usually a chronic condition, with periods where the disease is active, and other periods where it is not. The initial goal of treatment is to help patients enter remission, which is when there is an absence of signs or symptoms related to the disease. This phase of treatment is called "induction." Doctors will then transition a patient to "maintenance" treatment, which is to help keep the disease in remission.

During treatment, doctors will:  

• Keep track of your symptoms and any evidence of inflammation to see how well the treatment is working
• Use blood and urine (pee) tests to keep track of how well your kidneys are working

GPA symptoms can come back or new symptoms can arise after being in remission. This is often called a "relapse". It would be treated similarly to the original medications you were given. 

What type of doctors treat GPA?

GPA affects different areas of your body, so you may be treated by a team of specialists, such as:

• Rheumatologists – doctors who treat the immune system and diseases of the muscles and bones, such as arthritis
• Nephrologists – kidney doctors  
• Pulmonologists – lung and respiratory (breathing) system doctors  
• Otolaryngologists – ear, nose and throat doctors
• Dermatologists – skin doctors
• Neurologists – doctors who treat the nervous system

How will I know if I have GPA?

There is no one test for GPA. Many symptoms of GPA are similar to those caused by other health problems. To help make a diagnosis, doctors will look at your medical history, run specific tests and do a physical exam to check for skin rashes and signs of nerve damage.  

Common tests include:  

• Urine (pee) tests to check for red blood cells and protein
• Blood tests, such as ANCA testing  
• Imaging tests, such as X-rays or CT scans, to check for lung damage and swelling of the blood vessels

If your doctor thinks you may have GPA, they may recommend a biopsy (tissue sample) of your kidneys or skin to confirm a diagnosis. 

Can I prevent GPA? 

You cannot prevent GPA. If you have GPA, talk with your doctor about how to manage your symptoms and prevent further damage to your kidneys from occurring.  

If you and your doctor find your GPA before it damages your kidneys, you can help prevent kidney damage:  

• Take your medicines as your doctor tells you  
• Go to your doctor visits  
• Get any tests your doctor recommends  
• Talk to your doctor about any new or worsening symptoms
• Incorporate lifestyle changes like following a healthy eating plan, being active, limiting alcohol, or quitting smoking or using tobacco.

Where can I learn more about GPA?

If you or a loved one has been diagnosed with GPA learn more and connect with others by visiting the Vasculitis Foundation.