A critical appraisal of guidelines and recommendations for the management of ANCA-associated vasculitis

We aimed to appraise 11 currently available guidelines for ANCA-associated vasculitis (AAV), identify potential gaps, and provide recommendations for improvement.

The guidelines were appraised using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) instrument consisting of 23 appraisal criteria/items across six domains (D). Four reviewers independently appraised guidelines and scaled domain scores were calculated.

The average score of all guidelines was 4.5 ± 0.8 out of 7.0. Agreement between the reviewers was excellent. British Society for Rheumatology 2025 guidelines scored highest (5.8/7.0), being the only guideline unanimously recommended for use. In 'D1/Scope and purpose', all guidelines achieved high scores. In 'D2/Stakeholder involvement', major gaps were identified in the inclusion of the views and preferences of the target population (2c), with few guidelines including patient representatives, addressing patient participation, or considering patient views. In 'D3/Rigour of development', all but two guidelines received high scores. Implementation of the procedure for updating the guidelines (3 h) and for external review (3 g) was addressed poorly. In 'D4/Clarity of presentation', all guidelines except one received a high score. 'D5/Applicability' had the lowest scores due to gaps in the criteria for monitoring/auditing guideline implementation (5d), resource/cost implications (5c), lack of advice/tools for implementation (5a), and inadequate addressing of facilitators/barriers in guideline application (5a). 'D6/Editorial independence' was addressed well.

Several aspects of AAV guidelines could be improved, including the inclusion of patient views and preferences, the processes for translating guidelines into clinical practice, and clearer criteria for monitoring guideline implementation and uptake.