Understand the Impact of ANCA-Associated Vasculitis on the Body

ANCA-Associated Vasculitis: How It Can Impact the Body

This visual guide shows how the autoimmune condition can affect various areas of your body.

A nti-neutrophil cytoplasmic autoantibodies (ANCA)–associated vasculitis, or AAV, is a group of autoimmune diseases that can damage small blood vessels in many organs. “It most often involves the skin, kidneys, lungs, sinuses, and airways,” says Shubhasree Banerjee, MD, an assistant professor of rheumatology at Penn Medicine in Philadelphia. It can also cause generalized symptoms, such as decreased appetite, fatigue, fever, overall weakness, and weight loss, she says.

Knowing how AAV can affect your body and health is key to managing this chronic condition. Here’s a closer look at how AAV can affect various areas.

How AAV Causes Inflammation in Blood Vessels

In AAV, the immune system makes autoantibodies (malfunctioning antibodies that attack the body’s healthy tissues) called ANCAs, which attack neutrophils, a type of white blood cell that helps your body fight infection.

When ANCAs attach to neutrophils, the neutrophils release substances that cause inflammation in small blood vessels.

 “This inflammation is what causes the symptoms of the disease,” says Dr. Banerjee. It causes the walls of the blood vessels to swell and thicken, which makes it harder for blood to flow through as it should. Over time, this inflammation can damage the walls of the blood vessels.
Neutrophils can move through the damaged walls, causing inflammation in surrounding tissues. This can attract even more neutrophils to the area, contributing to additional inflammation and damage to the blood vessels.

Possible Effects of AAV on the Body: A Head-to-Toe Guide

AAV can affect different parts of the body, leading to a range of symptoms. “The symptoms of ANCA-associated vasculitis can vary widely from person to person, depending on which organs are affected,” says Banerjee. And some symptoms may be detected only through lab results. 

Why Early Diagnosis Matters

If left untreated, ANCA-associated vasculitis can potentially be life threatening and cause permanent damage. According to Banerjee, untreated AAV can lead to complications such as:

  • Airway damage requiring a permanent breathing tube
  • Damage to the nasal septum that can change the shape of the nose
  • Hearing loss
  • Kidney failure that may require long-term dialysis
  • Nerve damage resulting in permanent weakness in different body parts

“Early kidney involvement may cause no symptoms and is often detected only through urine testing, which may show protein, blood, or abnormal cells,” says Banerjee. “As the disease progresses, kidney function may decline, leading to elevated creatinine levels in the blood. In severe cases, kidney failure can occur, and dialysis may be required.”

How AAV Treatment Slows or Prevents Damage to the Body

The goal of ANCA-associated vasculitis treatment is to reduce inflammation and calm the overactive immune system, so it stops attacking your body.

Early diagnosis and prompt treatment can help reduce the risk of serious complications and prevent permanent organ damage.

Treatment is generally divided into two parts: the induction and maintenance phases.

AAV Treatment: Induction Phase

The induction phase focuses on stopping active inflammation and bringing the disease into remission.

This is typically achieved with medications, including chemotherapy and immunotherapy drugs and corticosteroids, but individual treatment plans will depend on factors such as disease severity and type of AAV.

Glucocorticoids, also called steroids, are anti-inflammatory medications that act quickly to suppress the immune system. In the induction phase, moderate-to-high doses of corticosteroids are often prescribed, along with cyclophosphamide (Cytoxan), a chemotherapy drug, or rituximab (Rituxan), a monoclonal antibody that works by blocking immune cells that produce harmful ANCA antibodies. For milder cases of AAV, steroids may be combined with methotrexate (Rheumatrex, Trexall), another immunosuppressant.

Another drug approved by the U.S. Food and Drug Administration to treat AAV is avacopan (Tavneos). “It works by blocking a part of the immune system called the complement pathway, which activates the white blood cells to release inflammatory substances,” says Banerjee. “By turning off this cycle, avacopan reduces further inflammation in the blood vessels.”

For severe cases of AAV, where there may be bleeding in the lungs or severe kidney damage, the doctor may order plasmapheresis. This procedure involves running your blood through a machine that removes antibodies, including ANCA autoantibodies, and then returning the blood to your body.

AAV Treatment: Maintenance Phase

Once inflammation is under control, you can move into the maintenance phase of treatment. The goal of this phase is to keep ANCA-associated vasculitis in remission and prevent relapses.

During this phase, your doctor may lower the dose of corticosteroids and add medications such as rituximab, methotrexate, or azathioprine (Imuran).

Throughout treatment, your doctor will monitor you with regular follow-up exams. They’ll also order blood, urine, or other tests to assess inflammation and ANCA antibody levels and look for further organ damage.

Communication with your doctor is an important part of AAV maintenance therapy, says Banerjee, who advises keeping your doctor informed about how symptoms affect you and how you feel overall. “As I always tell my patients, ‘You give me the most valuable information about your disease. Your symptoms and how you feel are the key to knowing whether your vasculitis is well controlled or in remission.’”

The Takeaway

  • ANCA-associated vasculitis is a group of autoimmune conditions that cause inflammation and swelling in small blood vessels in organs throughout the body.
  • Symptoms can vary widely, depending on which organs are affected, and range from mild to potentially life threatening, if vital organs are affected.
  • The goal of treatment, which includes the induction and maintenance phases, is to reduce inflammation and suppress the overactive immune system.
EDITORIAL SOURCES
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Resources
  1. Autoantibodies. Cleveland Clinic. September 4, 2024.
  2. Murphy S. ANCA Vasculitis. University of North Carolina Kidney Center. September 2018.
  3. McClure ME et al. What Is ANCA?: Anti-Neutrophil Cytoplasm Antibodies (ANCA). Vasculitis UK.
  4. Cyclophosphamide. MedlinePlus. February 15, 2025.
  5. Guillevin L. Advances in the Maintenance of ANCA Vasculitis Remission. Best Practice & Research Clinical Rheumatology. March 2023.
  6. Qasim A et al. ANCA-Associated Vasculitis. StatPearls. August 31, 2024.
Beth Biggee

Beth Biggee, MD

Medical Reviewer

Beth Biggee, MD, is owner and practitioner of Lifestyle and Integrative Rheumatology, a holistic direct specialty care practice in North Andover, Massachusetts. She offers whole-person autoimmune care, lifestyle medicine, and holistic integrative consults.

She has over 20 years of experience in rheumatology and holds board certifications in rheumatology and integrative and lifestyle medicine. Dr. Biggee brings a human-centered approach to wellness rather than focusing solely on diseases.

Biggee graduated cum laude with a bachelor's degree from Canisius College, and graduated magna cum laude and as valedictorian from SUNY Health Science Center at Syracuse Medical School. She completed her internship and residency in internal medicine at Yale New Haven Hospital, her fellowship in rheumatology at Tufts–New England Medical Center, and her training in integrative rheumatology at the University of Arizona Andrew Weil Center for Integrative Medicine.

Following her training, she attained board certification in rheumatology and internal medicine through the American Board of Internal Medicine, board certification in integrative medicine through the American Board of Physician Specialties, and accreditation as a certified lifestyle medicine physician through the American College of Lifestyle Medicine. She is certified in Helms auricular acupuncture and is currently completing coursework in the Aloha Ayurveda integrative medicine course for physicians.

In prior roles, Biggee was medical director and integrative rheumatologist at Rheumission, a virtual integrative rheumatology practice, and she also provided healthcare wellness consulting for Synergy Wellness Center in Hudson, Massachusetts. Biggee taught as an assistant clinical professor of medicine at Mary Imogene Bassett Hospital (an affiliate of Columbia University). She was also clinical associate of medicine at Tufts University School of Medicine and taught Introduction to Clinical Medicine for medical students at Tufts. She was preceptor for the Lawrence General Hospital Family Medicine Residency.

Biggee has published work in the Annals of the Rheumatic Diseases, Arthritis & Rheumatology, Current Opinion in Rheumatology, Medicine and Health Rhode Island, and the Field Guide to Internal Medicine.

Erin-Coakley-article

Erin Coakley

Author

Erin guides editorial direction and content for custom projects. Before joining Everyday Health, she was associate editor at dLife, an online resource for people managing diabetes. Erin majored in English with a minor in psychology at Stonehill College in Easton, Massachusetts. Outside of work she enjoys reading, going to concerts, traveling, and working out. She recently did 867 pushups in an hour to help send children with serious illnesses to camp.