BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis

Abstract

Scope and purpose of the guideline: The ANCA-associated vasculitides (AAVs) are heterogeneous, multisystem disorders characterized by inflammation and necrosis of small and medium blood vessels with unknown aetiology. Three distinct clinico-pathological syndromes have been identified: granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. The Chapel Hill Consensus Conference (CHCC) in 2012 updated the definitions, however, there are still no validated diagnostic criteria. The aim of this document is to provide guidelines for the management of adults with AAV. The target audience is rheumatologists, nephrologists, general physicians, specialists, trainees and nurse practitioners. The guideline does not cover the management of other systemic vasculitides or the treatment of children. This is a short summary of the guideline. The full guideline is available as supplementary material, available at Rheumatology Online. For definitions of levels of evidence and recommendation strength see Tables 1 and 2. Guideline for the management of adults with AAV: We have produced evidence-based recommendations for treatment, giving a grade of recommendation (from A to D) and an algorithm to illustrate the approach to the management of a patient with newly diagnosed AAV.