Advancements in ANCA-Associated Vasculitis Treatment
Managing ANCA-Associated Vasculitis

What Does Treatment for ANCA-Associated Vasculitis (AAV) Look Like Today?

What Does Treatment for ANCA-Associated Vasculitis (AAV) Look Like Today?
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Published on March 6, 2026
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There are two main goals of antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis (AAV) treatment.

The first goal is to bring the condition into remission by controlling active inflammation that can cause permanent organ damage. The second is to stay in remission and prevent flare-ups.

Today, these goals are more achievable, thanks to advancements in AAV treatment. “It’s a dramatically different landscape now than before,” says Robert Spiera, MD, a rheumatologist at Hospital for Special Surgery in New York City. “Our patients do exponentially better than 50 years ago. We are better at monitoring patients and better at how we use the medicines, [and we can shift the focus to] minimizing damage.” With better treatment options today — and more on the horizon — many people with AAV can live much longer, healthier lives.

ANCA-Associated Vasculitis Treatment for Reaching Remission

Your individual AAV treatment plan will depend on factors such as the severity of the disease, the type of AAV you have, and which areas of your body are affected.

 Some of the treatments you may be prescribed include:

Rituximab

For severe disease that may involve symptoms that affect the kidneys and cause arthritis, bleeding in the lungs, or otherwise high disease activity, rituximab is one of the common medicines prescribed, says Dr. Spiera. It’s often used in conjunction with glucocorticoids.

Rituximab targets and destroys B cells, the immune cells that produce the abnormal antibodies. When B cells are eliminated, ANCA levels and the inflammation process are reduced.

This medication is administered intravenously. Before the infusion, patients are given glucocorticoids to minimize the side effects of rituximab infusion–related reactions, such as breathing problems and chills. The infusion itself can take four to six hours.

Side effects may include:

  • An allergic reaction to the infusion
  • Bacterial, fungal, or viral infections
  • Less protective effects for vaccines
  • Low immunoglobulin levels

Cyclophosphamide

Prescribed for people with severe, life-threatening disease activity, this chemotherapy drug may be prescribed instead of or in combination with rituximab. Like rituximab, it is prescribed along with steroids (glucocorticoids).

 Cyclophosphamide works by stopping the production of abnormally overactive immune cells that are responsible for inflammation.

Cyclophosphamide can be given intravenously or in pill form. It’s very important to drink plenty of fluids when taking this medication, as it can cause serious irritation if it stays in the bladder too long.

Side effects may include:

  • An increased risk of infection
  • Bleeding in the bladder
  • Hair loss
  • Lower white blood cell count
  • Reproductive risks
  • Secondary cancers

Methotrexate

For non-organ-threatening and non-life-threatening disease, methotrexate — a disease-modifying antirheumatic drug that’s often used as a first-line treatment for psoriasis, rheumatoid arthritis, and other autoimmune conditions — can be given orally or subcutaneously (under the skin) to treat AAV. Like rituximab and cyclophosphamide, it is an immunosuppressant and anti-inflammatory drug that interferes with the production of immune cells.

Side effects may include:


  • Fatigue
  • Hair loss
  • Liver abnormalities
  • Mouth sores
  • Stomach and bowel problems

Glucocorticoids

These steroids — usually prednisone — are used with rituximab, cyclophosphamide, and methotrexate. Steroids act as anti-inflammatories and can reduce the damage caused by the overactive immune system. The dosages vary, but the goal is to prescribe the lowest effective dose to avoid bad side effects. Long-term use is associated with significant toxicity. “We try to get patients off within six months,” says Spiera.

Side effects may include:

  • Cognitive changes
  • Difficulty sleeping
  • High blood sugar
  • Hypertension
  • Increased risk of infection
  • Muscle weakness
  • Osteoporosis
  • Skin fragility

Avacopan

Recently, avacopan has been paired with rituximab, with the benefit of dialing back the length of steroid treatment. Avacopan works by blocking a damaging inflammatory molecule called C5a from attaching to receptors on a type of white blood cell called a neutrophil. This blocking action reduces inflammation and consequent blood vessel and kidney damage.

Research shows the steroid-sparing benefits of avacopan can also greatly improve quality of life in people with AAV.

Spiera says trials are under way to see if a rituximab-glucocorticoid-avacopan combination can help prevent damage to the sinuses and nasal passages. “That’s a major determinant for quality of life, because people incur so much damage in the sino-nasal domain,” he says.

Side effects may include:


  • An allergic reaction (shortness of breath, facial swelling, dizziness)
  • Infection
  • Liver problems
  • Stomach upset

Mycophenolate

This immunosuppressant is sometimes used for non-organ-threatening and non-life-threatening disease. It may be selected carefully for certain subgroups of people who have vasculitis without severe or rapid risk of renal disease. Given orally, mycophenolate has a higher relapse rate for some types of AAV.

 Side effects may include:

  • Risk of infection
  • Risks to reproductive health (including a high rate of miscarriage)
  • Stomach upset


ANCA-Associated Vasculitis Treatment for Maintaining Remission

The second part of the strategy for managing ANCA-associated vasculitis is a medication regimen to help you stay in remission. In some cases maintenance can span years, so the goal is to minimize the toxicity and side effects of the medications.

Spiera says an important part of remission maintenance is being vigilant about follow-up visits, so you can be evaluated for any changes in disease activity and medication side effects. Staying on top of appointments will help prevent relapse.

According to research, rituximab is emerging as a better option for remission than some of the other medications available, says Spiera.

Other treatments currently in use for maintaining remission include:

  • Azathioprine But recent studies have shown that rituximab is superior to azathrioprine for preventing relapse.

  • Methotrexate It’s not prescribed as much, because rituximab has shown better results.

  • Mycophenolate This is also sometimes used in maintenance therapy.

Overall, the decision to use azathioprine, methotrexate, or mycophenolate as maintenance therapy will depend on individual factors and be decided by a team of vasculitis experts, including a rheumatologist, pulmonologist, nephrologist, and other specialists.

Look to the Future of Treatment

The good news is that there are even more treatments being studied, with a focus on minimizing damage to the body caused by inflammation of the blood vessels. “Your expectation can be that you can live a very full life,” says Spiera.

One of the new therapies being looked at is CAR T-cell therapy, in which your own T cells are used to target and eliminate the rogue B cells that are overproducing the autoantibodies. This therapy, which is still in the early stages of investigation (in animals), could potentially take the place of immunosuppressants one day.

The Takeaway

  • The goal of AAV treatment is to first get the disease in remission and then help you stay in remission.
  • You may be prescribed a combination of medications that, at various times, can include steroids, rituximab, mycophenolate, methotrexate, and cyclophosphamide, depending on the severity of the disease and whether you’re in remission.
  • The future of treatment looks promising, with targeted CAR T-cell therapy and potential new drugs aimed at reducing the side effects of medication.
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
  1. ANCA-Associated Vasculitis. Cleveland Clinic. October 15, 2025.
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  3. Rituximab (Intravenous Route). Mayo Clinic. February 1, 2026.
  4. Cyclophosphamide (Oral Route, Intravenous Route). Mayo Clinic. February 1, 2026.
  5. Treatment Recommendations for Active Severe, or Active Nonsevere GPA/MPA. Vasculitis Foundation. February 2024.
  6. Methotrexate (Oral Route). Mayo Clinic. February 1, 2026.
  7. Gaspar DD et al. ANCA-Associated Vasculitis (AAV). Rare Disease Advisor. July 1, 2025.
  8. Aldhuaina K et al. Efficacy and Safety of Avacopan in the Treatment of ANCA-Associated Vasculitis: A Systematic Review and Meta-Analysis . BMC Rheumatology. October 3, 2025.
  9. Tavneos (Avacopan). GoodRx. May 20, 2025.
  10. Jones RB et al. Mycophenolate Mofetil Versus Cyclophosphamide for Remission Induction in ANCA-Associated Vasculitis: A Randomised, Non-Inferiority Trial. Annals of the Rheumatic Diseases. March 2019.
  11. Mycophenolate Mofetil (Oral Route). Mayo Clinic. February 1, 2026.
  12. Alberici F et al. Treatment Goals in ANCA-Associated Vasculitis: Defining Success in a New Era. Frontiers in Immunology. June 12, 2024.
  13. Chalkia A et al. ANCA-Associated Vasculitis–Treatment Standard. Nephrology Dialysis Transplantation. November 8, 2023.
  14. Smith RM et al. Rituximab Versus Azathioprine for Maintenance of Remission for Patients With ANCA-Associated Vasculitis and Relapsing Disease: An International Randomised Controlled Trial. Annals of the Rheumatic Diseases. July 2023.
  15. Dörte L et al. CD19-Targeting CAR T Cells Protect From ANCA-Induced Acute Kidney Injury. Annals of the Rheumatic Diseases. April 2024.
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Beth Biggee

Beth Biggee, MD

Medical Reviewer
Member of American College of Lifestyle Medicine, Castle Connolly Top Doctor

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.

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Nina Wasserman

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Nina Wasserman is a journalist with more than a decade of experience interviewing people and writing on a variety of topics, including health, medicine, business, and faith, as well as human interest stories. Wasserman also home-schools her two children in New Jersey and teaches writing to middle school students. Her passion is foraging for mushrooms and edible plants in the woods, a practice that contributes to her health and wellness.

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