Advancements in ANCA-Associated Vasculitis Treatment

Posted by AweSamo March 7, 2026
What Does Treatment for ANCA-Associated Vasculitis (AAV) Look Like Today?

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
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