NIAID Research Informs Guideline for Treating People with ANCA-Associated Vasculitis

NIAID Now | July 14, 2021

Image of vascular branches in the retina

Projected image (focus stacked) of the vascular branches in the retina at the back of the eye.

Credit: Bryan William Jones

A study funded by the National Institute of Allergy and Infectious Diseases (NIAID) contributes to the evidence for a new guideline on how to induce remission and treat relapse of a rare, severe autoimmune disease called ANCA-associated vasculitis. In people with this disease, antibodies attack immune cells called neutrophils, causing inflammation in small- to medium-sized blood vessels. This inflammation leads to potentially life-threatening organ damage, particularly in the lungs and kidneys. ANCA stands for anti-neutrophil cytoplasmic antibody.

The recommendations are part of a larger set of new, evidence-based guidelines for the diagnosis, treatment and management of patients with systemic vasculitis, which includes ANCA-associated vasculitis. The American College of Rheumatology developed the guidelines in partnership with the Vasculitis Foundation and published them last week in the journals Arthritis & Rheumatology and Arthritis Care & Research.

The NIAID study found in 2013 that giving the drug rituximab to people with ANCA-associated vasculitis once weekly for a month provided the same benefits as 18 months of daily immunosuppressive therapy with cyclophosphamide followed by azathioprine, the standard of care for many years. Rituximab depletes the body’s supply of cells thought to be responsible for producing ANCA and thus is much more targeted than nonspecific immunosuppressive therapy, which also has potentially severe, long-term side-effects.

The new guideline recommends that doctors use rituximab as a first-line drug to induce and maintain remission of active, severe ANCA-associated vasculitis. It also recommends that doctors use rituximab to re-induce remission of ANCA-associated vasculitis in patients who had responded to the drug previously but were not receiving it to maintain remission and relapsed with severe disease.


U Specks et al. Efficacy of remission induction regimens for ANCA-associated vasculitis. New England Journal of Medicine. DOI: 10.1056/NEJMoa1213277 (2013).

SA Chung et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis & Rheumatology DOI: 10.1002/art.41773 (2021).

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