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Vasculitis
Original research
Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1—treatment of granulomatosis with polyangiitis and microscopic polyangiitis
  1. Jan Henrik Schirmer1,
  2. Beatriz Sanchez-Alamo2,3,
  3. Bernhard Hellmich4,
  4. David Jayne5,
  5. Sara Monti6,
  6. Raashid Ahmed Luqmani7 and
  7. Gunnar Tomasson8
  1. 1Clinic for Internal Medicine I, Rheumatology and Clinical Immunology, University Medical Center Schleswig-Holstein Campus Kiel, Kiel, Germany
  2. 2Nephrology, Hospital Universitario del Sureste, Arganda del Rey, Madrid, Spain
  3. 3Nephrology, Skåne University Hospital, Lund, Sweden
  4. 4Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken Kirchheim/Teck, University Tübingen, Kirchheim-Teck, Germany
  5. 5Department of Medicine, University of Cambridge, Cambridge, UK
  6. 6Department of Internal Medicine and Therapeutics, University of Pavia; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
  7. 7Oxford NIHR Biomedical Research Centre, Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK
  8. 8Faculty of Medicine, University of Iceland, Landspitali University Hospital, Reykjavik, Iceland
  1. Correspondence to Dr Jan Henrik Schirmer; janhenrik.schirmer{at}uksh.de

Abstract

Objective To summarise and update evidence to inform the 2022 update of the EULAR recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitis (AAV).

Methods A systematic literature review (SLR) was performed to identify current evidence regarding treatment of AAV. PubMed, EMBASE and the Cochrane library were searched from 1 February 2015 to 25 February 2022. The evidence presented here is focused on the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.

Results 3517 articles were screened and 175 assessed by full-text review. Ninety articles were included in the final evidence synthesis. Cyclophosphamide and rituximab (RTX) show similar efficacy for remission induction (level of evidence (LoE) 1a) but RTX is more effective in relapsing disease (LoE 1b). Glucocorticoid (GC) protocols with faster tapering result in similar remission rates but lower rates of serious infections (LoE 1b). Avacopan can be used to rapidly taper and replace GC (LoE 1b). Data on plasma exchange are inconsistent depending on the analysed trial populations but meta-analyses based on randomised controlled trials demonstrate a reduction of the risk of end-stage kidney disease at 1 year but not during long-term follow-up (LoE 1a). Use of RTX for maintenance of remission is associated with lower relapse rates compared with azathioprine (AZA, LoE 1b). Prolonged maintenance treatment results in lower relapse rates for both, AZA (LoE 1b) and RTX (LoE 1b).

Conclusion This SLR provides current evidence to inform the 2022 update of the EULAR recommendations for the management of AAV.

  • rituximab
  • cyclophosphamide
  • systemic vasculitis
  • granulomatosis with polyangiitis

Data availability statement

All data relevant to the study are included in the article or uploaded as supplementary information.

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/rmdopen-2023-003082

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    Data availability statement

    All data relevant to the study are included in the article or uploaded as supplementary information.

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    Footnotes

    • JHS and BS-A are joint first authors.

    • JHS and BS-A contributed equally.

    • Contributors BS-A and JHS conducted the SLR under supervision of the methodologists (RAL and GT). DJ, BH, GT, SM and RAL were members of the steering committee and provided substantial methodological advice. JHS and BS-A drafted the first version of the manuscript and subsequent revisions. All authors were involved in the formulation and discussion of the evidence and reviewed the manuscript and evidence tables. The final version of the manuscript was approved by all authors. The guarantor for this manuscript is JHS.

    • Funding This project was funded by EULAR. DJ was supported by the NIHR Cambridge Biomedical Research Centre.

    • Competing interests JHS received research grants from the John Grube Foundation and the Deutsche Gesellschaft für Rheumatologie (German Society for Rheumatology). BH received speaker fees and/or consultancies from AbbVie, Amgen, AstraZeneca, BMS, Boehringer, Chugai, GSK, InflaRx, Janssen, MSD, Pfizer, Novartis, Phadia, Roche and Vifor. DJ received speaker fees and/or consultancies from Amgen, AstraZeneca, BMS, Boehringer, Chemocentryx, GSK, InflaRx, Janssen, Novartis, Roche, UCB and Vifor. RAL received speaker and/or consulting fees and/or grants from AbbVie, BMS/Celgene, Chemocentryx, Chugai, GSK, InflaRx, Pfizer Global, Roche and Vifor.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.