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REVIEW article

Front. Immunol.

Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders

Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1655326

This article is part of the Research TopicCommunity Series: Systemic Vasculitis: Advances in Pathogenesis and Therapies Volume IIView all 6 articles

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: Insights into Relapse Risk and Future Management Directions

Provisionally accepted
  • 1University of Brescia, Brescia, Italy
  • 2Renal Unit, Royal Berkshire Hospital, London Road, Reading, Berkshire, UK, Reading, United Kingdom
  • 3Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany, Lubeck, Germany
  • 4Molecular Immunity Unit, Department of Medicine, University of Cambridge, Cambridge UK, Cambridge, United Kingdom
  • 5Rheumatology Unit, Department of Medicine DIMED, University of Padua, Padua, Italy, Padoa, Italy
  • 6CSL Vifor, Glattbrugg, Switzerland, Glattbtugg, Switzerland
  • 7Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia and Università di Modena e Reggio 13 Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy
  • 8Department of Rheumatology, Skåne University Hospital, Lund, Sweden and Clinical Sciences, 15 Rheumatology, Lund University, Lund, Sweden, Lund, Sweden

The final, formatted version of the article will be published soon.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has a relapsing-remitting course and, even with the availability of effective maintenance therapies such as rituximab, relapse rates remain high. Relapse is associated with the accrual of organ damage stemming from both the underlying disease and from the effects of AAV treatments; thus, early detection and proactive prevention are crucial. AAV study populations typically include mixed cohorts of patients with newonset and relapsing disease. Although data specifically addressing re-induction of remission after relapse are limited, available evidence suggests high remission rates when rituximab is combined with glucocorticoids. However, the balance between effective disease control and the potential treatmentrelated side effects must be carefully considered, and new therapeutic options may help improve this tradeoff. The aim of this review is to explore what is known about relapse risk and relapse management while considering emerging pathogenic and therapeutic paradigms.

Keywords: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, AAV relapse, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), remission re-induction

Received: 27 Jun 2025; Accepted: 18 Aug 2025.

Copyright: © 2025 Alberici, Flossmann, Lamprecht, Loudon, Padoan, Popov, Salvarani and Mohammad. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Federico Alberici, University of Brescia, Brescia, Italy

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