Vasculitides: Proposal for an integrated nomenclature

The vasculitides form a heterogeneous group of systemic diseases that differ in etiology, histological patterns, and, consequently, clinical significance and prognosis but are traceable to the same pathological event, namely, vessel wall inflammation. The clinical heterogeneity among these diseases, together with yet unknown pathogenetic mechanisms for many of them, creates difficulties in the early diagnosis and correct management of affected patients. Therefore, several groups of investigators have elaborated nomenclatures to set some order in the definition and grouping of the vasculitides. The two main naming systems used for decades, i.e., the Fauci nomenclature and the 1994 Chapel Hill Consensus Conference (CHCC) nomenclature, were recently superseded by a revised CHCC nomenclature published in 2012. The aim of that revision was to update the names and definitions of the vasculitides and to include novel forms, considering the advances in knowledge made since the first consensus conference was held. Here, we critically discuss the 2012 CHCC nomenclature in light of the earlier naming systems and raise some concerns in how several vasculitides were grouped. On the basis of this analysis, we propose an integrated nomenclature that we believe will have a more direct impact in the clinic, perfectly aware that any redefinition may present contradictions.