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  1. J. Schirmer1,
  2. B. Sanchez-Alamo2,
  3. S. Monti3,
  4. B. Hellmich4,
  5. D. Jayne5
  1. 1University Medical Center Schleswig-Holstein, Clinic for Internal Medicine I, Section for Rheumatology, Kiel, Germany
  2. 2Lund University, Skåne University Hospital, Nephrology, Lund, Sweden
  3. 3University of Pavia, Fondazione IRCCS Policlinico S. Matteo, Rheumatology, Pavia, Italy
  4. 4University of Tübingen, Medius Kliniken, Internal Medicine, Rheumatology and Immunology, Kirchheim-Teck, Germany
  5. 5University of Cambridge, Addenbrooke’s Hospital, Department of Medicine, Cambridge, United Kingdom


Background The 2008 and 2016 European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of ANCA-associated vasculitis (AAV)1,2 have supported clinicians with comprehensive recommendations for the treatment of patients with AAV in daily practice. During the past 5 years, the publication of several high-impact randomized-controlled studies have further improved the standard of care of AAV.

Objectives The aim of this systematic review was to collect evidence supporting the 2022 update the AAV management recommendations.

Methods The recommendations were developed based on an evidence-based approach as outlined in the 2014 EULAR standardized operating procedures (SOP)3. 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 a search strategy consisting of keywords identifying treatment-related topics of interest was created based on the PICO questions. Aspects of drug treatment and other therapeutic interventions in AAV were included in the search, with a focus on remission induction, maintenance treatment and steroid sparing protocols. Outcomes such as survival, remission/relapses, infectious complications and malignancies were also covered. PubMed (Medline), Embase and the Cochrane Library databases were searched for articles providing data on the search questions. Abstracts of the annual meetings of EULAR, ACR, ERA-EDTA, ASN and the Vasculitis and ANCA Workshops were also screened, but restricted to randomized controlled clinical trials (RCTs).

After deduplication publications were sorted by title and abstract first. There was full text review for articles eligible after title/abstract screening. The data were extracted from included articles and grouped according to the PICO questions. Data extraction results were collected in evidence tables.

The Cochrane revised tool for assessing risk of bias for RCTs (RoB2), ROBINS-1 for observational studies and AMSTAR II for meta-analyses were used for bias assessment. Evidence was categorized based on the GRADE system as per EULAR SOP3.

Results Based on the results of the Delphi, 11 topics related to therapeutic interventions were identified that were transformed into PICO questions (Table 1). Other items that received lower scores were added in the format of subquestions. Based on these research questions, search strings for the SLR were created.

Table 1.

Key topics of interest for treatment strategies identified in the Delphi exercise grouped according to the PICO format

The SLR was still ongoing at the time this abstract has been written and results of the SLR will be presented at the meeting.

Conclusion This SLR identified recent developments affecting key areas of AAV treatment, that provide systematic evidence to inform the 2022 update of the EULAR recommendations for the management of AAV, which will also be presented at this meeting.

References [1]Mukhtyar C, et al. EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2008;68:310-317.

[2]Yates M et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Sep;75(9):1583-94

[3]van der Heijde D et al. 2014 Update of the EULAR standardised operating procedures for EULAR-endorsed recommendations. Ann Rheum Dis. 2015 Jan;74(1):8-13.

Acknowledgements The project is funded by EULAR.

Disclosure of Interests Jan Schirmer: None declared, Beatriz Sanchez-Alamo: None declared, Sara Monti: None declared, Bernhard Hellmich Speakers bureau: Abbvie, BMS, Chugai, GSK, MSD, Novartis, Pfizer, Roche, Vifor, Consultant of: Boehringer, BMS, Chugai, GSK, InflaRx, Novartis, Roche, Vifor, David Jayne Speakers bureau: Vifor, Consultant of: Astra-Zeneca, Boehringer, BMS, Chemocentryx, Chugai, GSK, Novartis, Roche

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