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Update on Maintenance Therapies for ANCA-Associated Vasculitis

  • Vasculitis (L Barra, Section Editor)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of review

Over the past decades, maintenance therapy in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) has greatly evolved, in a smooth step-wise manner. Refinements are still needed, and more changes will likely occur soon. Here, we review the historical steps that led to the current standard for maintenance therapy and the results of some recent studies that could reshape it further.

Recent findings

Although older drugs, such as azathioprine or methotrexate, still have a place as possible maintenance agents for AAV, rituximab (RTX) was found the most effective at preventing relapses in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), in combination, at least initially, with low-dose glucocorticoids (GCs). The optimal regimen of maintenance therapy with RTX and its duration remain to be determined. Ideally, it would be based on a patient-individualized approach. Pharmacodynamic and pharmacogenetic studies may eventually help optimize therapy. In parallel, newer agents, such as avacopan, may further revolutionize how we treat GPA and MPA, by replacing or greatly limiting the use of GCs for both induction and maintenance of remission. Fewer studies have investigated eosinophilic granulomatosis with polyangiitis, but more data have been produced recently for the use of mepolizumab or rituximab to maintain good control of asthma and rhino-sinusitis.

Summary

With more refined approaches and new therapies in AAV, relapse-free survival at 5 and 10 years has significantly improved over the last 20 years. However, many questions about the optimal maintenance treatment remain unanswered, and new questions have emerged with new therapeutic options. There is still a need for further studies to improve disease control and achieve sustained and possibly drug-free remission.

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  1. Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, 60 Murray Street, Ste 2-220, Box 8, Toronto, Ontario, M5T 3L9, Canada

    Christian Pagnoux MD, MSc, MPH & Aurore Fifi-Mah MD, FRCPC

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Christian Pagnoux declares having received fees for serving on advisory boards of Chemocentryx, GlaxoSmithKline, InflaRx, Astra-Zeneca, Sanofi, and Hoffman-LaRoche and lecture fees and educational grant support from Hoffman-La Roche and GlaxoSmithKline.

Aurore Fifi-Mah declares having received fees for serving on advisory boards from Hoffman-La Roche and Sanofi and a research grant from Hoffman-La Roche.

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Pagnoux, C., Fifi-Mah, A. Update on Maintenance Therapies for ANCA-Associated Vasculitis. Curr Treat Options in Rheum 7, 112–133 (2021). https://doi.org/10.1007/s40674-021-00176-z

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