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 recommendations for the accurate diagnosis, monitoring and for the management of the long-term complications of patients with ANCA-associated vasculitis (AAV). Since the publication of the last EULAR guidelines, several high-impact research articles have provided further evidence to improve diagnosis and monitoring of AAV-patients.
Objectives The aim of this systematic review was collecting evidence supporting the 2022 update of the AAV management recommendations.
Methods The recommendations were developed based on the 2014 EULAR standardized operating procedures (SOP)3. Areas of interest were adopted from the 2016 recommendations and updated by identifying additional items through a Delphi exercise. Key questions were framed in the PICO (Population, Intervention, Comparator, Outcome) format. Keywords identifying topics of interest for the diagnosis and follow up were gathered based on the PICO questions and then incorporated into a search string. A systematic literature research (SLR) was performed according to the EULAR SOP. 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 first sorted by title and abstract and then full text review was done for eligible articles. 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, QUADAS II for diagnostic accuracy 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, 3 topics related to diagnosis and follow-up were identified that were transformed into PICO questions: the impact of tissue biopsies and positive ANCA testing to support the clinical diagnosis of AAV and the impact of clinical parameters and biomarkers on disease-related outcomes and treatment-related adverse events (Table 1). Other items that received lower scores in the Delphi exercise were added in the format of subquestions (e.g. diagnostic imaging). Based on these research questions, search strings for the SLR were created.
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 diagnosis and follow-up. The results of this SLR 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 Mukhtyar C, et al. EULAR Recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2008;68:310-317.
Yates M et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Sep;75(9):1583-94
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 Beatriz Sanchez-Alamo: None declared, Jan Schirmer: 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, Takeda
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