The European Alliance of Associations for Rheumatology (EULAR) task force has conducted a systematic literature review and expert survey with the aim of updating the 2016 EULAR recommendations for managing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

The updated guidance, published in the Annals of Rheumatic Diseases, comprises 4 overarching principles and 17 recommendations.

The new recommendations were drafted using standard EULAR operating procedures by a task force of 20 clinical experts in AAV from 15 European countries and the United States. Two patient representatives were also included. Fourteen key questions in AAV management were identified and assessed in a systematic literature review of articles published since February 2015.

“Recent landmark studies on the role of plasma exchange (PLEX), standardization of glucocorticoid (GC) dosing, use of rituximab (RTX) for maintenance therapy, C5a receptor (C5aR)-targeted and anti-interleukin 5 (IL-5) therapy in [eosinophilic granulomatosis with polyangiitis] make this an opportune time to update the 2016 guidelines,” the authors wrote.

“These recommendations address the diagnosis and treatment of adult patients with AAV and are intended to give advice to clinicians, other health professionals, pharmaceutical companies, and regulatory organizations.”

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A face-to-face meeting was held with the task force members to vote on the draft recommendations, and agreement on each recommendation and the 4 overarching principles was submitted anonymously by email after the meeting.

The new recommendations include biopsies and ANCA testing for AAV diagnoses, and for remission induction, a combination of high-dose GCs with either RTX or cyclophosphamide. GCs should be tapered within 4 to 5 months to a target dose of 5 mg of prednisone per day. RTX is recommended for remission maintenance of granulomatosis with polyangiitis or microscopic polyangiitis.

The authors note that although some of the new, substantially revised recommendations are based on low-quality evidence, the agreement level among the task force representatives was consistently high.


Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. Published online March 16, 2023. doi:10.1136/ard-2022-223764