What Is ANCA-Associated Vasculitis (AAV)?
Published on February 4, 2026
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ANCA-associated vasculitis (AAV) refers not to a single health condition, but rather a group of rare autoimmune diseases in which the immune system mistakenly attacks small blood vessels. ANCA stands for antineutrophil cytoplasmic antibodies — immune system proteins that attack white blood cells called neutrophils. Vasculitis is an inflammation of the blood vessels.
AAV causes blood vessels to swell and thicken, reducing blood flow to many parts of the body, including various organs, nerves, the skin, and joints. There is no cure for AAV, but early treatment can prevent serious damage to your organs and help you reach remission, in which there’s little to no AAV-related inflammation in your body.
Types of ANCA-Associated Vasculitis
AAV is categorized into three types.
- Granulomatosis With Polyangiitis (GPA) Once called Wegener’s granulomatosis, GPA commonly affects the small vessels in the respiratory tract (particularly the sinuses, nose, throat, and lungs) and kidneys. In GPA, clusters of inflammatory cells called granulomas form in blood vessels and organs.
- Eosinophilic Granulomatosis With Polyangiitis (EGPA) Previously known as Churg–Strauss syndrome, EGPA affects people with a history of severe asthma or allergies and is marked by unusually high levels of eosinophils (a type of white blood cell) in the blood or tissues. EGPA most often affects the lungs but can also involve other organs in the body.
- Microscopic Polyangiitis (MPA) MPA almost always affects the kidneys, and also often affects the lungs (though not as frequently as GPA), nerves, skin, and joints. Unlike GPA and EPGA, MPA does not cause clusters of inflammatory cells (granulomas).
Among the three types of AAV, GPA is the most common, followed by MPA.
Signs and Symptoms of ANCA-Associated Vasculitis
People with AAV frequently experience generalized symptoms such as:
- Fatigue
- Body aches
- Joint and muscle pain
- Fever
- Decreased appetite
- Unexplained weight loss
A wide variety of other symptoms are also possible, depending on which organs or parts of the body are affected, such as:
- Difficulty breathing or shortness of breath
- Cough (possibly with blood)
- Chest pain, which may worsen while inhaling
- Sinus pain, pressure, or congestion
- Sinus infections
- Nose bleeds
- Crusting around the nose
- Red, irritated eyes
- Eye inflammation such as conjunctivitis (pink eye)
- Blurred vision and vision changes
- Tinnitus (ringing in the ears)
- Ear infection or inflammation
- Hearing loss
- Blood in the urine
- Foamy urine (caused by protein)
- High blood pressure (new or worsened)
- Numbness, tingling, or weakness in different areas of the body (peripheral neuropathy)
- Skin rash, especially on the extremities, with small, raised, reddish-purplish spots
- Skin sores or ulcers
- Heart palpitations
Causes and Risk Factors of ANCA-Associated Vasculitis
AAV develops when malfunctioning immune cells attach to neutrophils (a type of white blood cell) and make them attack small vessels, causing inflammation. It’s not exactly known what causes this to happen, but several factors may be involved, including:
- Various genetic factors, such as those associated with the protein alpha 1 antitrypsin, the proteinase 3 (PR3) gene, or the major histocompatibility complex family of genes
- Bacterial infections with Staphylococcus aureus or viral infections with hepatitis C virus, Epstein-Barr virus, cytomegalovirus, or parvovirus
- Environmental exposure to pollutants or chemical compounds such as silica (found in sand, soil, and rock), paint thinners, and pesticides
- Certain medications to treat hyperthyroidism, hypertension, and bacterial infections, such as propylthiouracil, carbimazole, methimazole (Tapazole), hydralazine (Apresoline), and minocycline (Minocin)
AAV most often develops in people between ages 45 and 60, and it is slightly more common among males.
How Is ANCA-Associated Vasculitis Diagnosed?
There is no single test to diagnose AAV. Instead, healthcare providers make the diagnosis based on your medical history, family history, symptoms, physical examination, laboratory tests, imaging studies, and sometimes biopsies (taking a tissue sample to examine under a microscope).
A critical part of the diagnostic process involves having a detailed conversation about your symptoms, including when they started, how they feel, whether they come and go, and which activities, if any, make them worse. Be thorough when discussing your medical history, as some medications and health conditions are associated with AAV.
Various tests can help diagnose AAV:
- Blood and urine tests identify ANCA antibodies, look for inflammation, check kidney function, and detect blood or protein in the urine. While blood tests can detect antibodies associated with AAV, it’s not a surefire way to diagnose AAV. Some people with AAV test negative for ANCA antibodies (for example, about 60 percent of people with EGPA are ANCA-negative) and some other conditions (such as lupus) can cause a positive ANCA test result.
- Imaging studies, such as chest X-rays or computed tomography (CT) scans, can determine lung involvement and rule out infection- or cancer-related causes for your symptoms.
- Biopsy of an affected organ, such as the kidney, lung, or sinus, is the most reliable way to confirm vasculitis.
Treatment and Medication Options for ANCA-Associated Vasculitis
Treatment for AAV has improved dramatically over the years. It aims to control inflammation and push the condition into remission — and then maintain remission.
The exact treatment and medications you receive depend on multiple factors, including your AAV type and severity, how fast your disease is progressing, and which organs and tissues are affected.
Induction Therapy
The first phase of AAV treatment focuses on rapidly controlling inflammation to prevent organ damage and induce remission. This typically includes:
- High-dose corticosteroids
- cyclophosphamide (Cytoxan), a chemotherapy agent that’s been used longer for AAV than any other medication (besides steroids)
- rituximab (Rituxan), a medication that targets antibodies and appears to be as effective as cyclophosphamide and results in fewer side effects
- methotrexate or mycophenolate mofetil (CellCept or Myfortic), used in non-life-threatening cases
- Plasma exchange in severe cases, especially those involving the kidneys
In recent years, the U.S. Food and Drug Administration (FDA) approved new targeted therapies for AAV, including:
- avacopan (Tavneos), which blocks the neutrophil receptor (preventing antibodies from attaching to them), for GPA and MPA as an adjunctive or additional treatment
- mepolizumab (Nucala), which targets a protein that promotes the growth and survival of eosinophils (a type of white blood cell), for EGPA
- benralizumab (Fasenra), which also works to reduce the number of eosinophils, for EGPA
Maintenance Therapy
Once remission is achieved, lower-intensity treatment helps prevent relapse. Options include these immunosuppressant medications:
- rituximab
- azathioprine or methotrexate (only in the case of mild kidney damage, or eGFR greater than 60 mL/min/1.73m2)
Lifestyle Changes and Prevention of ANCA-Associated Vasculitis
There is no known way to prevent AAV from developing, but adequate treatment and maintenance can help prevent serious complications and remission relapse.
Certain lifestyle changes can help you better manage or live with AAV, improving your quality of life. These include:
- Physical therapy, which may help reduce fatigue and improve physical and mental well-being
- A healthy, balanced diet tailored to your specific needs, such as reduced protein and potassium if you have kidney failure, reduced salt intake if you have high blood pressure, and increased calcium while taking steroids
- Not smoking
- Reducing or eliminating alcohol intake
- Maintaining a physically active lifestyle to reduce inflammation, manage stress, maintain strength and bone density, and improve sleep
- Connecting with mental health professionals, support groups, and advocacy organizations to improve and maintain emotional well-being
- Avoiding dust, fumes, and other asthma-triggering substances in the workplace and home
- Managing asthma symptoms if you have EGPA
ANCA-Associated Vasculitis Prognosis
AAV is a chronic, relapsing condition that requires lifelong care and maintenance. Severe AAV can be fatal if left untreated.
With modern therapy, most patients achieve remission. But remission relapse is common, especially with GPA (more than 50 percent experience relapse within five years).
In a European study of long-term outcomes in people with AAV, the median survival time after diagnosis was about 18 years. In another, smaller study, survival rates at one, three, and five years after diagnosis were 80 percent, 67 percent, and 56 percent, respectively.
Successful pregnancies are possible with careful planning (conception during remission is best) and pregnancy-safe medications.
Complications of ANCA-Associated Vasculitis
Potential complications from AAV include:
- Bleeding in the lungs
- Scarring of the lungs
- Nerve damage
- Kidney failure
- Heart failure
- Cardiovascular disease, such as stroke
- High blood pressure
- Mouth ulcers
- Infections related to immunosuppressive therapy
While treatment is critical to survival, it also carries risks of complications, including:
- Cancer
- Bone marrow failure
- Diabetes
- Osteoporosis
- Infertility
- Bladder inflammation
The Takeaway
- ANCA-associated vasculitis is rare but treatable, especially with early diagnosis.
- It can cause a wide range of symptoms depending on AAV type and the organs affected.
- There is no cure for AAV, but modern immunosuppressive therapies have dramatically improved outcomes, and most people achieve remission.
- AAV requires long-term management with medication, monitoring, and lifestyle support.
FAQ
Is AAV curable?
No, but long-term remission is common with treatment.
Is AAV hereditary?
AAV is not passed down in families, but certain genetic factors are associated with it.
Can I get pregnant with AAV?
Yes, successful pregnancies are possible with careful planning (conception during remission is best) and pregnancy-safe medications.
Does a positive ANCA antibody test result mean I have AAV?
Not necessarily. Other health conditions can result in a positive ANCA test result — and some people with AAV test negative for ANCA antibodies.
Is there a specific diet for AAV?
There is no AAV-specific diet, but you should avoid excessive eating and may need to make dietary adjustments for your specific situation, such as reducing protein and potassium intake if your kidneys are affected.
Resources We Trust
- Cleveland Clinic: Pregnancy in Vasculitis: Issues to Consider
- Mayo Clinic: Vasculitis
- American Lung Association: Learn About Eosinophilic Granulomatosis With Polyangiitis (EGPA)
- UNC Kidney Center: ANCA Vasculitis
- Vasculitis Foundation: Education
EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
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- Eosinophilic Granulomatosis With Polyangiitis (EGPA, Formerly Churg-Strauss Syndrome). Cleveland Clinic. July 25, 2024.
- Microscopic Polyangiitis (MPA). Cleveland Clinic. December 9, 2025.
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Meet Our Experts
Alexa Meara, MD
Medical Reviewer
Alexa Meara, MD, is an assistant professor of immunology and rheumatology at The Ohio State University. She maintains a multidisciplinary vasculitis clinic and supervises a longitudinal registry of lupus nephritis and vasculitis patients. Her clinical research is in improving patient–physician communication. She is involved in the medical school and the Lead-Serve-Inspire (LSI) curriculum and serves on the medical school admissions committee; she also teaches multiple aspects of the Part One curriculum. Her interests in medical-education research include remediation and work with struggling learners.
Dr. Meara received her medical degree from Georgetown University School of Medicine in Washington, DC. She completed her internal medicine training at East Carolina University (ECU) at Vidant Medical Center in Greenville, North Carolina, then spent two more years at ECU, first as chief resident in internal medicine, then as the associate training program director for internal medicine. She pursued further training in rheumatology at The Ohio State University in Columbus, completing a four-year clinical and research fellowship there in 2015.
Joseph Bennington-Castro
Author
Joseph Bennington-Castro is a science writer based in Hawaii. He has written well over a thousand articles for the general public on a wide range topics, including health, astronomy, archaeology, renewable energy, biomaterials, conservation, history, animal behavior, artificial intelligence, and many others.
In addition to writing for Everyday Health, Bennington-Castro has also written for publications such as Scientific American, National Geographic online, USA Today, Materials Research Society, Wired UK, Men's Journal, Live Science, Space.com, NBC News Mach, NOAA Fisheries, io9.com, and Discover.