Background/Purpose: Since the publication of the 2016 European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of ANCA-associated vasculitis (AAV), the publication of several high-impact randomized-controlled studies improved the standard of care of AAV. The aim of this systematic review was to collect evidence supporting the 2022 update the EULAR recommendations for the management of AAV.

Methods: The recommendations were developed based on an evidence-based approach as outlined in the EULAR standardized operating procedures (SOP). Areas of interest were adopted from the 2016 recommendations and updated by identifying additional topics through a Delphi process. Key questions were framed in the PICO (Population, Intervention, Comparator, Outcome) format and 3 search strategies (for treatment, diagnostic procedures and aspects of general management) were created based on the PICO questions. PubMed, Embase and the Cochrane Library databases were searched. After deduplication publications were sorted by title and abstract first and subsequentally assessed in full text if eligible after title/abstract screening. The data were extracted from included articles according to PICO questions and collected in evidence tables.

Results: Based on the results of the Delphi, apart from domains of general management, 11 topics related to therapeutic interventions and 3 topics related to diagnostic and follow-up were identified that were transformed into PICO questions.

After deduplication 3510, 4130 and 4205 abstracts were identified for the areas of treatment, diagnosis/follow-up and general management, respectively. Other sources as congress abstracts were additionally searched.

Key findings discovered by the systematic literature research (SLR) include an update of diagnostic studies on biopsies and ANCA testing. It summarizes randomized-controlled trials and meta-analyses covering remission induction treatment with cyclophosphamide, rituximab and mycophenolate, the role of plasma exchange, steroid sparing protocols including the use of avacopan, duration of remission maintenance treatment with conventional immunosuppressives and rituximab. New data are available for the role of mepolizumab, rituximab, azathioprine and other treatment regimens in the management of Eosinophilic Granulomatosis with Polyangiitis.

Conclusion: This SLR identified recent developments affecting key areas of AAV management, that provide systematic evidence to inform the 2022 update of the EULAR recommendations for the management of AAV.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-systematic-literature-review-informing-the-2022-update-of-the-eular-recommendations-for-the-management-of-anca-associated-vasculitis/