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.e60dc2a1-f33c-4a05-9b50-8e3e8e59762930abcfd5-8be2-466f-a451-a4a66f104e04e60dc2a1-f33c-4a05-9b50-8e3e8e597629469dd86a-a7f5-4f49-a0c2-bdf648ddbafb 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.e60dc2a1-f33c-4a05-9b50-8e3e8e5976295359d56f-9180-4e51-8d94-e7d5d1f6048d 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 Types 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.e60dc2a1-f33c-4a05-9b50-8e3e8e597629b4959a4e-2c52-4523-bf3a-5368582595b7 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.e60dc2a1-f33c-4a05-9b50-8e3e8e597629db626c81-8546-483c-af87-16ecfb14177a 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).e60dc2a1-f33c-4a05-9b50-8e3e8e5976294bbee106-8782-4ab4-a84a-80f549ced54fe60dc2a1-f33c-4a05-9b50-8e3e8e59762966c06910-fa8c-433e-a8e6-567ba8274fa3 Among the three types of AAV, GPA is the most common, followed by MPA.e60dc2a1-f33c-4a05-9b50-8e3e8e5976294bbee106-8782-4ab4-a84a-80f549ced54f
Signs and Symptoms of ANCA-Associated Vasculitis Symptoms People with AAV frequently experience generalized symptoms such as:e60dc2a1-f33c-4a05-9b50-8e3e8e5976299b5c2663-75af-426f-94fc-8a138529c9c8e60dc2a1-f33c-4a05-9b50-8e3e8e5976296687a040-4b36-4d7f-a156-67e91d183866 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:e60dc2a1-f33c-4a05-9b50-8e3e8e5976296687a040-4b36-4d7f-a156-67e91d183866 Difficulty breathing or shortness of breath Cough (possibly with blood) Chest pain, which may worsen while inhalinge60dc2a1-f33c-4a05-9b50-8e3e8e5976299b5c2663-75af-426f-94fc-8a138529c9c8 Sinus pain, pressure, or congestion Sinus infections Nose bleeds Crusting around the nosee60dc2a1-f33c-4a05-9b50-8e3e8e597629b4a0f69e-3d4b-42db-ab7d-8e1526e5bac4 Red, irritated eyes Eye inflammation such as conjunctivitis (pink eye) Blurred vision and vision changese60dc2a1-f33c-4a05-9b50-8e3e8e59762981151238-2fa7-429c-9254-eadb86871935 Tinnitus (ringing in the ears)e60dc2a1-f33c-4a05-9b50-8e3e8e597629555aeaa7-aa34-4600-ba0a-822e26d03b73 Ear infection or inflammation Hearing loss Blood in the urine Foamy urine (caused by protein) High blood pressure (new or worsened)e60dc2a1-f33c-4a05-9b50-8e3e8e597629973f0aa8-da94-440e-9bfb-e572ef4c76df 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 palpitationse60dc2a1-f33c-4a05-9b50-8e3e8e5976299b5c2663-75af-426f-94fc-8a138529c9c8
Causes and Risk Factors of ANCA-Associated Vasculitis Causes 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:e60dc2a1-f33c-4a05-9b50-8e3e8e59762953fc9d87-34d4-414c-9ac8-f300761c4f53 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 pesticidese60dc2a1-f33c-4a05-9b50-8e3e8e5976298fafc1f6-1f83-4f85-ab59-69d113e646f2 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.e60dc2a1-f33c-4a05-9b50-8e3e8e59762953fc9d87-34d4-414c-9ac8-f300761c4f53
How Is ANCA-Associated Vasculitis Diagnosed? Diagnosis 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).e60dc2a1-f33c-4a05-9b50-8e3e8e5976294fba27b3-4ccf-40f5-a5f3-3739c4903604 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.e60dc2a1-f33c-4a05-9b50-8e3e8e597629ae943e12-f9cf-42e7-a748-486eb03d5e96 Various tests can help diagnose AAV:e60dc2a1-f33c-4a05-9b50-8e3e8e597629ae943e12-f9cf-42e7-a748-486eb03d5e96e60dc2a1-f33c-4a05-9b50-8e3e8e597629ab1562f0-5877-49c5-9069-da590f7a8c50 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.e60dc2a1-f33c-4a05-9b50-8e3e8e597629ae943e12-f9cf-42e7-a748-486eb03d5e96 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 Treatment for AAV has improved dramatically over the years. It aims to control inflammation and push the condition into remission — and then maintain remission.e60dc2a1-f33c-4a05-9b50-8e3e8e597629eab7fca9-4eca-4623-b4cd-e7d1432de4fc 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.e60dc2a1-f33c-4a05-9b50-8e3e8e597629157bba95-cfda-41cb-9b5d-c3eef57b310e Induction Therapy The first phase of AAV treatment focuses on rapidly controlling inflammation to prevent organ damage and induce remission. This typically includes:e60dc2a1-f33c-4a05-9b50-8e3e8e59762955bb53ca-4990-425a-be1a-e38dc77d75c0 High-dose corticosteroids cyclophosphamide (Cytoxan), a chemotherapy agent that’s been used longer for AAV than any other medication (besides steroids)e60dc2a1-f33c-4a05-9b50-8e3e8e5976296a6c9f49-a827-46f8-8189-4d1c9c13c22d 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 e60dc2a1-f33c-4a05-9b50-8e3e8e597629eab7fca9-4eca-4623-b4cd-e7d1432de4fc 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 treatmente60dc2a1-f33c-4a05-9b50-8e3e8e597629b7d3d035-a375-4cf7-95f3-8b839c3e99ac mepolizumab (Nucala), which targets a protein that promotes the growth and survival of eosinophils (a type of white blood cell), for EGPAe60dc2a1-f33c-4a05-9b50-8e3e8e5976293480e1c1-57b2-4173-ba5a-b26eb956da0c benralizumab (Fasenra), which also works to reduce the number of eosinophils, for EGPAe60dc2a1-f33c-4a05-9b50-8e3e8e597629256d97e4-64b7-4353-8ed8-03147cfb59fb Maintenance Therapy Once remission is achieved, lower-intensity treatment helps prevent relapse. Options include these immunosuppressant medications:e60dc2a1-f33c-4a05-9b50-8e3e8e59762955bb53ca-4990-425a-be1a-e38dc77d75c0 rituximab azathioprine or methotrexate (only in the case of mild kidney damage, or eGFR greater than 60 mL/min/1.73m 2 )
Lifestyle Changes and Prevention of ANCA-Associated Vasculitis Lifestyle Changes There is no known way to prevent AAV from developing, but adequate treatment and maintenance can help prevent serious complications and remission relapse.e60dc2a1-f33c-4a05-9b50-8e3e8e597629d548d19a-002e-4580-90e7-afbaae8a1423 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-beinge60dc2a1-f33c-4a05-9b50-8e3e8e5976291deccc2f-a44a-47ea-90ec-93182dd9a801 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 steroidse60dc2a1-f33c-4a05-9b50-8e3e8e597629293cf9c9-33c4-4d08-a81a-ac710c8a9707 Not smokinge60dc2a1-f33c-4a05-9b50-8e3e8e59762985e0c5e8-59c7-46a1-b771-f9674e200c40 Reducing or eliminating alcohol intakee60dc2a1-f33c-4a05-9b50-8e3e8e59762914270253-5c65-432f-be25-506f3198860b Maintaining a physically active lifestyle to reduce inflammation, manage stress, maintain strength and bone density, and improve sleepe60dc2a1-f33c-4a05-9b50-8e3e8e5976297c55b190-b971-4e25-9dc3-4ed31683bca6 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 homee60dc2a1-f33c-4a05-9b50-8e3e8e59762918d2e953-98b0-4961-a584-7b835da904eb Managing asthma symptoms if you have EGPAe60dc2a1-f33c-4a05-9b50-8e3e8e59762918d2e953-98b0-4961-a584-7b835da904eb
ANCA-Associated Vasculitis Prognosis Prognosis and Outlook AAV is a chronic, relapsing condition that requires lifelong care and maintenance. Severe AAV can be fatal if left untreated.e60dc2a1-f33c-4a05-9b50-8e3e8e597629dcee0865-9a1f-4ac1-835c-bcd60e2b6e06 With modern therapy, most patients achieve remission.e60dc2a1-f33c-4a05-9b50-8e3e8e597629ff6bbb5e-f3ad-4920-a9be-5defcc2ddcdc But remission relapse is common, especially with GPA (more than 50 percent experience relapse within five years).e60dc2a1-f33c-4a05-9b50-8e3e8e597629ff6bbb5e-f3ad-4920-a9be-5defcc2ddcdc In a European study of long-term outcomes in people with AAV, the median survival time after diagnosis was about 18 years.e60dc2a1-f33c-4a05-9b50-8e3e8e5976292ea3a537-aa8f-418f-9206-17a384706f1e In another, smaller study, survival rates at one, three, and five years after diagnosis were 80 percent, 67 percent, and 56 percent, respectively.e60dc2a1-f33c-4a05-9b50-8e3e8e5976297641f1e5-19c1-4479-9095-a941c7008391 Successful pregnancies are possible with careful planning (conception during remission is best) and pregnancy-safe medications.e60dc2a1-f33c-4a05-9b50-8e3e8e59762974bd92b5-4256-4197-9c20-7d1a35a8110c
Complications of ANCA-Associated Vasculitis Complications Potential complications from AAV include:e60dc2a1-f33c-4a05-9b50-8e3e8e597629353f0aa0-9b7f-4dcc-85d3-94dc107e90b4e60dc2a1-f33c-4a05-9b50-8e3e8e597629b25c8855-726f-4ee0-9a21-7258edca1500 Bleeding in the lungs Scarring of the lungs Nerve damagee60dc2a1-f33c-4a05-9b50-8e3e8e597629353f0aa0-9b7f-4dcc-85d3-94dc107e90b4 Kidney failure Heart failure Cardiovascular disease, such as stroke High blood pressure Mouth ulcers Infections related to immunosuppressive therapye60dc2a1-f33c-4a05-9b50-8e3e8e59762969289872-2adf-4c41-8d6a-b9af60bfb803 While treatment is critical to survival, it also carries risks of complications, including:e60dc2a1-f33c-4a05-9b50-8e3e8e59762969289872-2adf-4c41-8d6a-b9af60bfb803e60dc2a1-f33c-4a05-9b50-8e3e8e597629b25c8855-726f-4ee0-9a21-7258edca1500 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.
Resources We Trust Cleveland Clinic: Pregnancy in Vasculitis: Issues to ConsiderMayo Clinic: Vasculitis American Lung Association: Learn About Eosinophilic Granulomatosis With Polyangiitis (EGPA)UNC Kidney Center: ANCA VasculitisVasculitis Foundation: Education
What Is ANCA-Associated Vasculitis (AAV)?
