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.