Executive summary: The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis

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Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) are heterogeneous, multisystem disorders characterized by inflammation and necrosis of small and medium-sized blood vessels with unknown aetiology. Three distinct clinico-pathological syndromes have been identified: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) associated with autoantibodies directed against neutrophil granular proteins, proteinase 3 and myeloperoxidase. These conditions are uncommon with incidence and prevalence rates of ∼25/million population and 200/million, respectively [1]. Despite significant advances in treatment, mortality rates remain elevated, 2.3 times that of the general population [2]. Early diagnosis, instituting appropriate immunosuppression swiftly, and limiting toxicity from treatment is key to mitigating mortality and damage from AAV. The current British Society for Rheumatology (BSR) Guideline for management of AAV was completed in 2014 and was an important step forward in the management of these complex disorders [3]. It provided a roadmap for best practice management aiming to harmonize treatment and investigation of AAV. Updating the guideline is now required to reflect important changes incorporating new studies and trials describing significant advances in treatment and new therapies. The 2014 guideline no longer reflects current best practice and does not reflect all the available high-quality evidence that underpins management of AAV. Additionally, in line with other BSR guidelines and equality considerations, the updated recommendations now include consideration of people all of ages affected by AAV with specific consideration of the relevance to children and adolescents including transition into adult services. These recommendations were produced by comparing other international society recommendations and updating the review for UK practice. They offer systematic and evidence-based recommendations to support UK clinicians in the management of AAV across the whole life course. The target readership is all clinicians, including primary care, involved in management of people with AAV, and all people living with AAV. Diagnosis, and investigation of systemic GPA and MPA or the management of disease or treatment-related chronic damage is not covered in these recommendations, as the guidance outlined in the BSR guideline 2014 remains current. Significant advances in classification have occurred since the 2014 BSR guideline was produced. However, the classification is not validated for diagnosis or clinical management and is not included in this current guideline [4]. These recommendations were commissioned by the BSR Guidelines Steering Group (GSG). A working group (WG) was created involving relevant stakeholders. Recommendations in this report were developed, where appropriate, using the BSR Creating Clinical Guidelines Protocol using AGREEII (Appraisal of Guidelines for Research and Evaluation II) methodology. The guideline protocol was not followed fully, in that the scope of the work was not published prior to the final literature review. The reason for this was that, at the time this work was commenced, no significant new evidence had been highlighted since publication of the ACR 2021, KDIGO 2021 and update 2024, EULAR 2022 and EESG 2024 guidelines [5–8], and the intention was to provide a timely update of the BSR guideline 2014 relevant to the UK clinical context. It is for this reason that this manuscript is described as ‘Management Recommendations’ rather than a ‘Guideline’. Development was overseen by the BSR Guideline Steering Group throughout. Following a virtual meeting of the full Working Group (WG), the scope of the project was agreed and grouped into domains. Small working groups for each domain were formed and developed initial recommendations for discussion by the full WG. These initial recommendations were then adapted over a series of virtual meetings of the full WG. Each suggested recommendation in the final document was evaluated by all members and subjected to a vote relating to strength of agreement on a scale of 1 [no agreement] to 100% [complete agreement]. The wording of each recommendation was revised until all members were satisfied that they would score at least 80%. In addition, and in accordance with the BSR protocol, accompanying each recommendation in parentheses is a statement reflecting the strength of recommendation and quality of supporting evidence. Assessment of supporting evidence quality in GRADE reflects confidence in the estimates of benefits, harms and burdens. These recommendations use three levels and a letter (A, B, C) to reflect high, moderate or low/very low quality of evidence. The content and wording of all recommendations were also discussed in order for strength of recommendation to be agreed upon with all members of the WG, assigned as strong (designated as 1) or weak (designated as 2). The final draft of the recommendations was submitted to the BSR Guidelines Steering Group for stakeholder and internal review and feedback. Systematic literature searches were performed separately for each domain by the four subgroups. Where topics or questions had already been considered in previously published international guidelines, the literature search was from December 2021, the end of the European League Against Rheumatology (EULAR) 2022 guideline literature search. For new topics not covered in the BSR guideline 2014 or the published international guidelines [EULAR 2022, American College of Rheumatology (ACR) 2021, Kidney Disease Improving Global Outcomes (KDIGO) 2021 or European Eosinophilic Granulomatosis with Polyangiitis (eGPA) Study Group (EESG) Nature Evidence Based Guideline 2024] [5–8] the literature search was from December 1990. The evidence was drawn from Medline and limited to English language publications. Key terms were agreed within the members of the domain working group. The domain review groups prepared a summary of the quality of evidence following the GRADE approach (https://www.gradeworkinggroup.org/) that informed group review and discussion of the draft recommendations. 1. All people with lived experience of active (newly diagnosed or relapsed) AAV should be considered as having potentially life- or organ-threatening disease (GRADE 1C, SoA 98%). 2a. All people with lived experience of active GPA or MPA should be assessed for induction of remission treatment with immunosuppressants combined with glucocorticoids (GC) or avacopan (GRADE 1A, SoA 99%). 2b. The recommended options for immunosuppression for remission induction of newly diagnosed GPA or MPA are intravenous pulsed cyclophosphamide (CYC) or rituximab (RTX) (GRADE 1A, SoA 98%). 2c. For active relapsing disease, treatment with RTX is preferred (GRADE 1B, SoA 97%). 2d. A combination of both CYC and RTX can be considered for organ-threatening or life-threatening disease (GRADE 2C, SoA 98%). 2e. Certain patients with active GPA or MPA, with no evidence of life- or organ-threatening disease, may be considered for alternative induction therapy with methotrexate (MTX) or mycophenolate mofetil (MMF) (GRADE 1A, SoA 96%). 3a. Active GPA or MPA and severe kidney involvement with creatinine >300 μmol/l should be considered for adjunctive plasmapheresis provided their risk of potential adverse events has been considered (GRADE 2B, SoA 96%). 3b. For children living with active GPA or MPA, there is insufficient data to routinely recommend plasmapheresis for severe renal involvement; this therefore should only be considered on a case-by-case basis after discussion with an expert centre (GRADE 2C, SoA 96%). 3c. Adjunctive plasmapheresis is not routinely recommended for pulmonary haemorrhage without severe kidney involvement (GRADE 1A, SoA 96%). 4a. In patients with organ- or life-threatening disease, we advocate treatment with oral GC at a starting dose

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.