Rituximab, Cyclophosphamide, and Corticosteroids for ANCA Vasculitis: The Good, the Bad, and the Ugly

Abstract

Backgrounds: ANCA-associated vasculitis (AAV) frequently present with a chronic relapsing course. Relapse leads to an increased need for therapeutic agents and consequent toxicity. Summary: When referring to the available options for the management of AAV, the efficacy of glucocorticoids (GCs) is unquestionable. However, similarly unquestionable are their side effects. It has been more than 40 years since the efficacy of cyclophosphamide (CYC) as an add-on therapy to GCs in the management of necrotizing vasculitis has been proven. At the same time, concerns about the devastating side effects related to a prolonged exposure to this agent were raised. Despite the well-known side effects, the management of AAV remained centred on CYC until the early 2000s, when the pilot data first supporting the anecdotal efficacy of rituximab (RTX) were reported. However, it was not until 2010 that the non-inferiority of RTX to CYC for remission-induction in AAVs was demonstrated in 2 randomized controlled trials. Key Messages: Treatment of AAV has improved over the last decade, and currently available strategies are able to induce remission in the majority of the cases. Herewith, we aim to critically review available evidence and to critically address the following question: How can we reduce the GCs use the management of patients with AAV? Novel strategies that avoid the toxicity associated with currently used agents should be the goal. Ideally, these approaches should be GC-free.

Journal

Journal ID (publisher-id): KBR

Journal ID (nlm-ta): Kidney Blood Press Res

Journal ID (doi): 10.1159/issn.1420-4096

Title: Kidney and Blood Pressure Research

Publisher: S. Karger AG

ISSN (Print): 1420-4096

ISSN (Electronic): 1423-0143

Publication date Subscription-year: 2020

Publication date (Print): December 2020

Publication date (Electronic): 12 October 2020

Volume: 45

Issue: 6

Pages: 784-791

Affiliations

Nephrology and Dialysis Unit & CMID (Center of Research of Immunopathology and Rare Diseases), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital of Turin and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

Author notes

*Dario Roccatello, Center of Research of Immunopathology and Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, San Giovanni Hospitall, Piazza del Donatore di Sangue 3, IT–10154 Torino (Italy), [e-mail]

Article

Publisher ID: 509608 Other ID: Kidney Blood Press Res 2020;45:784–791

DOI: 10.1159/000509608

PubMed ID: 33045710

Copyright statement: © 2020 The Author(s) Published by S. Karger AG, Basel

License:

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Page count

Figures: 2, Tables: 1, Pages: 8

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Categories

Subject: Review Article