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Skopis et al. Vessel Plus 2020;4:30 Vessel Plus
DOI: 10.20517/2574-1209.2020.42

Review Open Access

Maintenance with Rituximab in anti-neutrophil
cytoplasm antibody-associated vasculitis

Maria Skopis , Ayse Bag-Ozbek 2
1
1 Department of Internal Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
2 Department of Internal Medicine, Division of Rheumatology, Allergy and Immunology, SUNY Stony Brook University Hospital,
Stony Brook, NY 11794, USA.
Correspondence to: Dr. Ayse Bag Ozbek, Department of Internal Medicine, Division of Rheumatology, Allergy and Immunology,
SUNY Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USA. E-mail: abagozbek@gmail.com
How to cite this article: Skopis M, Bag-Ozbek A. Maintenance with Rituximab in anti-neutrophil cytoplasm antibody-associated
vasculitis. Vessel Plus 2020;4:30. http://dx.doi.org/10.20517/2574-1209.2020.42.
Received: 20 Aug 2020 First Decision: 9 Sep 2020 Revised: 21 Sep 2020 Accepted: 23 Sep 2020 Published: 16 Oct 2020

Academic Editor: Luca Quartuccio Copy Editor: Cai-Hong Wang Production Editor: Jing Yu

Abstract
Received: First Decision: Revised: Accepted: Published: x
Granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited anti-neutrophil cytoplasm antibody
Science Editor: Copy Editor: Production Editor: Jing Yu (ANCA) associated vasculitis are the main ANCA-associated vasculitidies (AAV). Multiple induction therapies for
AAV exist and have proven successful in achieving disease remission. Azathioprine and methotrexate have been
used to maintain remission of AAV, however, relapse rates and adverse effects with these medications remain
high. Rituximab (RTX), a B cell depleting monoclonal antibody, was shown to be safe and effective in maintaining
disease remission in AAV in early retrospective reviews. In 2014, the first randomized control trial to compare RTX
and azathioprine in maintenance therapy of newly diagnosed AAV (MAINRITSAN trial), revealed that patients
who received RTX after cyclophosphamide induction had higher rates of sustained remission, fewer adverse
effects and, better overall survival rates as compared to azathioprine. MAINRITSAN 2 revealed that patients
receiving tailored regimens of maintenance RTX received fewer infusions but did not have higher rates of relapse
than patients who received fixed dose therapy. The RITAZAREM trial conveyed that patients who experienced
AAV relapse after induction therapy that received induction and maintenance RTX were significantly less likely
to develop a relapse at 24 months vs . patients who received maintenance therapy with azathioprine. Overall,
these studies suggest that maintenance therapy with RTX represents an exceptional treatment option in patients
with AAV in terms of safety and efficacy, resulting in lower relapse rates and less drug toxicity than conventional
treatments. As a result, patients have fewer exposures to cytotoxic medications and thus, improved outcomes.

Keywords: Rituximab, ANCA vasculitis, maintenance therapy, B cell depletion, azathioprine, methotrexate

© The Author(s) 2020. Open Access This article is licensed under a Creative Commons Attribution 4.0
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