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

Abstract

ANCA-associated vasculitis (AAV) is comprised of three specific conditions: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Since the publication of the last British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV in 2014, a plethora of randomized controlled trials, additional research and recommendations have provided novel insights into how the management of AAV can be optimized, thus improving patient quality of life. The BSR AAV Working Group (WG) reviewed published guidelines, undertook a systematic literature review and utilized expertise from specialist vasculitis centres across the UK and patient representatives to formulate a list of 26 recommendations with corresponding strength of agreement (SOA) scores. Recommendations were updated from the published 2014 BSR and BHPR guideline. The 26 recommendations encompassed five key domains: 1. Treatment for GPA and MPA; 2. Management of subglottic stenosis and ear, nose and throat (ENT) manifestations of AAV; 3. Management and treatment for EGPA; 4. Service specifications; 5. Patient education and support. These recommendations provide an update on care delivery of AAV based on current evidence and specialist opinion. In addition, we have provided research and audit recommendations to support equitable access to care and improve health outcomes. The lay summary that accompanies this abstract can be found in Supplementary Data S1, available at Rheumatology online.

Keywords: ANCA vasculitis; treatment.

Graphical abstract

Figure 1.

GPA and MPA treatment algorithm. ^aA combination of CYC and RTX can be considered in life-threatening or organ-threatening disease. ^bFor patients with active relapsing disease, treatment with RTX is preferred (recommendation 2c). ^cCreatinine >300 µmol/l. ^dPLEX can be considered provided that the risk of adverse events has been weighed against the benefits. ^eThe recommended duration of avacopan use is 12 months as there is no data on its use beyond this (recommendation 6). ^fThe optimum duration of GC tapering is uncertain. A suggested tapering regime is outlined in Table 3 (recommendation 10). ^gMMF can be considered when there is contraindication or intolerance to RTX, AZA or MTX. ^hDecision making around the length of treatment should consider patient risk factors for relapse and infection as well as patient preferences. AZA: azathioprine; CYC: cyclophosphamide; GC: glucocorticoids; MMF: mycophenolate mofetil; MTX: methotrexate; PLEX: plasma exchange; RTX: rituximab

Figure 2.

Proposed EGPA management algorithm (adapted from latest EULAR recommendations with permission [10]). ^aRTX is only conditionally recommended in cases of confirmed vasculitic complications (as opposed to type-2 mediated inflammation) when CYC is less preferable (e.g., patients with childbearing potential, previous exposure to a large burden of cumulative CYC and/or strong patient preference). ^banti-IL-5/5R therapy is recommended if patient meets NICE TA criteria for any of the currently licensed clinical indications (e.g. severe eosinophilic asthma). ^cThere is limited data to support the benefit of AZA in EGPA, whilst no RCTs are available on the use of MTX or MMF. ^dGC should be tapered to the lowest possible effective dose whilst maintaining disease remission and considering patient-specific disease manifestations, comorbidities and preferences. A slow gradual taper below physiological doses (approximately prednisolone 3 mg daily) should be attempted in the majority of cases to allow adrenal recovery and avoid long-term adrenal insufficiency. ^eDependent on specialist assessment of individual disease manifestation(s), comorbidities and patient preferences. Anti-IL-5/5R: anti-interleukin-5/anti-interleukin-5 receptor biologics; AZA: azathioprine; GC: glucocorticoids; CYC: cyclophosphamide; EGPA: eosinophilic granulomatosis with polyangiitis; HES: hypereosinophilic syndrome; MMF: mycophenolate mofetil; MTX: methotrexate; RTX: rituximab