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.