Management of antineutrophil cytoplasmic antibody-associated vasculitis: a changing tide

Purpose of review: Antineutrophil cytoplasmic antibody associated vasculitis (AAV) is a group of autoimmune disorders of small blood vessels. While outcomes in AAV have improved with the use of glucocorticoids (GC) and other immunosuppressants, these treatments are associated with significant toxicities. Infections are the major cause of mortality within the first year of treatment. There is a move towards newer treatments with better safety profiles. This review reflects on recent advances in the treatment of AAV.

Recent findings: The role of plasma exchange (PLEX) in AAV with kidney involvement has been clarified with new BMJ guideline recommendations following the publication of PEXIVAS and an updated meta-analysis. Lower dose GC regimens are now standard of care. Avacopan (C5a receptor antagonist) was noninferior to a regimen of GC therapy and is a potential steroid-sparing agent. Lastly, rituximab-based regimens were noninferior to cyclophosphamide in two trials for induction of remission and superior to azathioprine in one trial of maintenance of remission.

Summary: AAV treatments have changed tremendously over the past decade with a drive towards targeted PLEX use, increased rituximab use and lower GC dosing. Striking a crucial balance between morbidity from relapses and toxicities from immunosuppression remains a challenging path to navigate.

Comments：

Antineutrophil cytoplasmic antibody associated vasculitis (AAV) is a group of autoimmune disorders of small blood vessels. While glucocorticoids (GC) and other immunosuppressants have improved outcomes, they are associated with significant toxicities, particularly infections which are a major cause of mortality within the first year of treatment. Recent advances in the treatment of AAV have focused on reducing the toxicity of existing treatments, and on exploring newer treatments with better safety profiles.

Recent findings have clarified the role of plasma exchange (PLEX) in AAV with kidney involvement, and lowered the dose of GC regimens. Avacopan, a C5a receptor antagonist, was found to be noninferior to GC therapy and may be a potential steroid-sparing agent. Lastly, rituximab-based regimens were found to be noninferior to cyclophosphamide in two trials for induction of remission and superior to azathioprine in one trial of maintenance of remission.

Overall, AAV treatments have undergone significant changes over the past decade, with a move towards targeted PLEX use, increased rituximab use, and lower GC dosing. The challenge remains striking a balance between preventing relapses and minimizing toxicities from immunosuppression.

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