Last updated: July 20, 2026
Medically reviewed by: NKF Patient Education Team
Learn about ANCA-associated vasculitis, how it affects the kidneys, available treatments, questions to ask your care team, and where to find support.
About ANCA-Associated Vasculitis (AAV)
ANCA-associated vasculitis (AAV) is a group of rare diseases that cause inflammation (swelling) in small blood vessels. AAV is considered an “autoimmune” disease, meaning your body’s immune system attacks your own healthy tissues by mistake, leading to organ damage. AAV can affect many parts of the body, including your kidneys, lungs, sinuses, ears, skin, nerves, and eyes.
When AAV affects the kidneys, it can cause “ANCA-associated glomerulonephritis”. This is also known as ANCA-GN or ANCA-related kidney disease. Inflammation from AAV can damage your kidneys’ glomeruli - the tiny filters that remove waste and extra fluid from your blood. This can make it harder for your kidneys to work properly.
Early diagnosis and treatment are important because untreated disease can lead to serious kidney or other organ damage.
Signs and Symptoms
General symptoms
Each person will have different symptoms, and they can appear fast or develop slowly. These can include:
- Feeling very tired or low energy (fatigue)
- Fever
- Weight loss without trying
- Loss of appetite (not feeling hungry)
- Muscle aches
- Joint pain
- Trouble concentrating
Kidney symptoms
Many people with ANCA-related kidney disease have no symptoms at first. As kidney damage worsens, symptoms may include:
- Swelling in your feet, ankles, legs, or around your eyes
- Foamy urine
- Blood in your urine (hematuria)
- High blood pressure
- Making less urine than usual
Other symptoms
Depending on the type of AAV you have, other symptoms can include:
- Stuffy or bloody nose
- Frequent sinus infections
- Ear pain or hearing changes
- Shortness of breath (trouble breathing)
- Skin rash
- Numbness or tingling in your hands or feet
- Eye pain or redness
Causes
The exact cause of AAV is not fully understood. Researchers believe it happens when the immune system becomes overactive and attacks small blood vessels. Several factors may play a role, including:
- Genetics (traits passed down through families)
- Environmental factors (like pollution in your air, water, or food)
- Certain infections
- Changes in the immune system
AAV is not contagious, meaning you can't catch it from someone else. Also, nothing you did caused the disease.
Types
There are three main types of AAV:
Microscopic Polyangiitis (MPA)
- Commonly affects the kidneys and lungs
- The most common cause of ANCA-related kidney disease
Granulomatosis with Polyangiitis (GPA)
- Previously called Wegener’s disease
- Often affects the sinuses, nose, ears, lungs, and kidneys
- Another common cause of ANCA-related kidney disease
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
- Previously called Churg-Strauss disease
- Often affects people with asthma or allergies
- Can affect the lungs, nerves, skin, and heart, and sometimes the kidneys
Complications
Without treatment, AAV can cause chronic kidney disease (CKD) and kidney failure. Every person’s experience is different. For some people, the journey progresses slowly, while others advance to kidney failure very quickly.
Other possible complications from AAV include:
- Bleeding in your lungs
- Breathing problems
- Hearing loss
- Nerve damage
- Vision problems
- Heart disease
Also, AAV treatments work by lowering immune system activity. So, another complication includes an increased risk of infections.
Emotional wellness
An AAV diagnosis can be scary and overwhelming. It is normal to feel worried, sad, angry, or unsure about what comes next. You do not have to face AAV alone. Your healthcare team, family, friends, and patient support groups can help. If worry, fear, or sadness start affecting your daily life, let your healthcare team know. You can also call or text 988 to reach the Suicide & Crisis Lifeline. Getting support for your emotional health is just as important as caring for your physical health.
Diagnosis
AAV can be difficult to diagnose because its symptoms may look like many other diseases. So, your healthcare team will likely need a combination of tests to get you the right diagnosis:
Blood Tests
- Antibodies linked to the disease, such as ANCA screen, Anti-PR3, and Anti-MPO
- Kidney function tests, like creatinine and estimated glomerular filtration rate (eGFR)
- Complete blood count, to check your red cells (carry oxygen) and white cells (fight infections)
- Tests for inflammation, like erythrocyte sedimentation rate (ESR) or C-reactive protein (hs-CRP)
Urine Tests
- Urinalysis to check for general signs of health issues, like blood or protein in your urine
- Urine albumin-creatinine ratio (uACR) to check for albuminuria, a common sign of kidney damage
- Urine protein-creatinine ratio (uPCR) may also be used. This test is similar to uACR. But it measures all the different proteins that may be in your urine instead of just albumin.
Imaging tests
- Chest X-ray, since AAV often affects the lungs too
- CT scan of your chest or sinuses
Kidney biopsy
A kidney biopsy is usually needed to confirm ANCA-related kidney disease. During this procedure, a small piece of your kidney is removed with a special needle and looked at under a microscope. The biopsy helps confirm the diagnosis, measure the amount of kidney damage, and guide treatment decisions.
Treatment
Overview
The goals of treatment are:
- Stop inflammation (swelling) in your kidneys and blood vessels
- Protect your organs
- Prevent future disease flare-ups
- Preserve kidney function
Treatment often happens in two phases:
1) controlling active disease (induction), and
2) preventing it from coming back (maintenance).
Medications
Some of the most common medicines for treating AAV are listed below. Most people will need a combination of medicines to help control the disease without lowering the immune system too much.
- Corticosteroids (steroids) work quickly to reduce swelling. They work very well for AAV but may cause side effects when used at high doses or for long periods of time. Examples include prednisone and prednisolone.
- Immunosuppressive medicines calm the immune system and help stop blood vessel damage. Examples include rituximab (Rituxan), cyclophosphamide (Cytoxan), azathioprine, and mycophenolate mofetil (CellCept or MMF).
- Complement inhibitors can also help reduce swelling caused by a part of the immune system. They are more targeted than steroids and may help some people lower their steroid dose. Examples include avacopan (Tavneos).
Depending on your condition, your healthcare team may also recommend other treatments, including:
- Medicines to help manage high blood pressure
- Medicines to protect your kidney function
- Vaccines to help prevent infections
- Antibiotics to prevent infection during treatment
Plasma exchange (plasmapheresis)
In some severe cases of AAV, your healthcare team may recommend plasma exchange. This is also called plasmapheresis (plaz-muh-fuh-REE-sis). This procedure filters your blood to remove harmful antibodies and other substances that may be causing inflammation (swelling). It is sometimes used when there is significant kidney damage or bleeding in the lungs. It is usually combined with other treatments above.
Nutrition
There is no special diet that cures AAV, but healthy eating can support your overall health and kidney function. Your healthcare team may recommend:
- Eating plenty of fruits and vegetables
- Choosing lean proteins, especially plant-based proteins
- Limiting highly processed foods
- Reducing sodium (salt) intake
- Staying hydrated
Nutrition recommendations are highly customized to your body’s specific needs and dietary preferences. So, work with a kidney dietitian to build a plan tailored to you.
Exercise
Physical activity can help improve your strength, energy levels, mood, and heart health. It can also be a great way to reduce stress. Aim for regular movement that fits your abilities, such as walking, swimming, cycling, stretching, or light strength training.
If you are having a disease flare, recovering from induction treatment, or have severe kidney disease, ask your healthcare team what level of activity is safe for you before starting a new exercise plan.
Preparing for your appointment
Questions to ask
- Is my kidney function stable, improving, or getting worse?
- What steps can I take to slow down the kidney disease process?
- What signs or symptoms should I watch for that could mean my disease is becoming active again?
- What should I do if I think I am having a flare or experiencing a medication side effect?
- What is my risk of developing kidney failure?
- What side effects should I watch for, and when should I call the clinic?
- Is it safe for me to become pregnant or father a child while taking my medications?
- How will we know if my treatment is working?
- Am I at higher risk of infection because of my treatment? If so, how can I reduce that risk?
Kidney health information, support, and professional resources
Learn more about kidney health
More education and information from NKF
- NKF Cares: Patient Information Help Line
- NKF KidneyCARE (Community Access to Research Equity) study
- NKF Kidney Learning Center
