Proposal for a more practical classification of antineutrophil cytoplasmic antibody-associated vasculitis

Abstract

The nomenclature for antineutrophil cytoplasmic antibody (ANCA)-associated kidney disease has evolved from honorific eponyms to a descriptive-based classification scheme (Chapel Hill Consensus Conference 2012). Microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis do not correlate with presentation, response rates and relapse rates as when comparing myeloperoxidase versus leukocyte proteinase 3. Here we discuss the limitations of the currently used classification and propose an alternative, simple classification according to (i) ANCA type and (ii) organ involvement, which provides important clinical information of prognosis and outcomes.