PMID- 37479496 OWN - NLM STAT- MEDLINE DCOM- 20231103 LR - 20231103 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 3 DP - 2023 Jul TI - 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. LID - 10.1136/rmdopen-2023-003082 [doi] LID - e003082 AB - 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. CI - (c) Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Schirmer, Jan Henrik AU - Schirmer JH AUID- ORCID: 0000-0001-5467-2783 AD - Clinic for Internal Medicine I, Rheumatology and Clinical Immunology, University Medical Center Schleswig-Holstein Campus Kiel, Kiel, Germany janhenrik.schirmer@uksh.de. FAU - Sanchez-Alamo, Beatriz AU - Sanchez-Alamo B AUID- ORCID: 0000-0002-7835-543X AD - Nephrology, Hospital Universitario del Sureste, Arganda del Rey, Madrid, Spain. AD - Nephrology, Skane University Hospital, Lund, Sweden. FAU - Hellmich, Bernhard AU - Hellmich B AUID- ORCID: 0000-0002-8014-1801 AD - Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken Kirchheim/Teck, University Tubingen, Kirchheim-Teck, Germany. FAU - Jayne, David AU - Jayne D AUID- ORCID: 0000-0002-1712-0637 AD - Department of Medicine, University of Cambridge, Cambridge, UK. FAU - Monti, Sara AU - Monti S AUID- ORCID: 0000-0002-1800-6772 AD - Department of Internal Medicine and Therapeutics, University of Pavia; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Luqmani, Raashid Ahmed AU - Luqmani RA AD - Oxford NIHR Biomedical Research Centre, Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK. FAU - Tomasson, Gunnar AU - Tomasson G AUID- ORCID: 0000-0002-1797-7091 AD - Faculty of Medicine, University of Iceland, Landspitali University Hospital, Reykjavik, Iceland. LA - eng GR - DH_/Department of Health/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 8N3DW7272P (Cyclophosphamide) RN - 4F4X42SYQ6 (Rituximab) RN - 0 (Glucocorticoids) SB - IM MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis MH - *Granulomatosis with Polyangiitis MH - Humans MH - *Microscopic Polyangiitis MH - *Cyclophosphamide/therapeutic use MH - *Rituximab/therapeutic use MH - Glucocorticoids MH - Remission Induction PMC - PMC10364171 OTO - NOTNLM OT - cyclophosphamide OT - granulomatosis with polyangiitis OT - rituximab OT - systemic vasculitis COIS- Competing interests: JHS received research grants from the John Grube Foundation and the Deutsche Gesellschaft fur 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. EDAT- 2023/07/22 10:42 MHDA- 2023/07/26 06:43 PMCR- 2023/07/21 CRDT- 2023/07/21 21:23 PHST- 2023/02/18 00:00 [received] PHST- 2023/05/16 00:00 [accepted] PHST- 2023/07/26 06:43 [medline] PHST- 2023/07/22 10:42 [pubmed] PHST- 2023/07/21 21:23 [entrez] PHST- 2023/07/21 00:00 [pmc-release] AID - rmdopen-2023-003082 [pii] AID - 10.1136/rmdopen-2023-003082 [doi] PST - ppublish SO - RMD Open. 2023 Jul;9(3):e003082. doi: 10.1136/rmdopen-2023-003082.