The management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has come a long way from the first postmortem descriptions of the diseases in the 1930s and 1950s1 Pivotal phases have been the introduction of glucocorticoids (GC) and cyclophosphamide (CYC), the association with ANCA in the 1980s, and consensus classification and nomenclature systems from 1990 onwards2.