A task force of the European Alliance of Associations for Rheumatology (EULAR) recently published an update to its 2016 recommendations for managing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

For the update, which was published in the Annals of the Rheumatic Diseases, the task force formulated 4 overarching principles and 17 recommendations.

“These recommendations address the diagnosis and treatment of adult patients with [ANCA-associated vasculitis] and are intended to give advice to clinicians, other health professionals, pharmaceutical companies and regulatory organisations,” the task force, led by Bernhard Hellmich, MD, of Medius Klinik Kirchheim, Teaching Hospital of the University of Tübingen in Germany, wrote.

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One overarching principle is that patients with ANCA-associated vasculitis should be offered the best care based on shared decision-making between the patient and the physician that takes into account efficacy, safety, and cost. A second principle is that patients should have access to education focusing on the burden of ANCA-associated vasculitis and its prognosis, key warning symptoms, and treatment (including treatment-related complications). The authors noted that this statement is unchanged from the 2016 update (formerly statement number 14) and has been recategorized as an overarching principle.

A third principle is that patients should be screened periodically for treatment-related adverse effects and comorbidities. “We recommend prophylaxis and lifestyle advice to reduce treatment-related complications and other comorbidities.”

In the fourth principle, the task force noted that ANCA-associated vasculitis cases are rare, heterogeneous, and potentially life- and organ-threatening “and thus require multidisciplinary management by centres with, or with ready access to, expertise in vasculitis.”

Among its 17 recommendations, the task force advocates biopsies and ANCA testing to assist in establishing a diagnosis of ANCA-associated vasculitis. The organization also recommends high-dose glucocorticoids combined with either rituximab or cyclophosphamide for remission induction in life-threatening or organ-threatening ANCA-associated vasculitis.

The task force recommends tapering of glucocorticoid doses to a target of 5 mg prednisolone equivalents per day within 4 to 5 months. Avacopan may be considered as part of a strategy to reduce exposure to glucocorticoids in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis. For patients with relapsing or refractory eosinophilic GPA, the task force recommends the use of mepolizumab.

“Given the complexity and variability of multiorgan involvement in [ANCA-associated vasculitis], we emphasise that these recommendations are not intended to propose a ‘one-size-fits-all’ strategy,” the authors wrote. “Comorbidities, the individual patient’s history, toxicities, local availability and costs of medication, and patient preferences should all be considered in the process of informed decision-making.”


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