Background The nomenclature established at the first International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides has been widely used since its publication in 1994 and has been cited approximately 2,000 times in the medical literature indexed in the MEDLINE database. Here we report revisions recommended by another International Chapel Hill Consensus Conference convened in 2011 to make improvements in the first nomenclature, including changes in names and definitions, and the addition of important categories of the vasculitides not previously included. A disease nomenclature system specifies the name that should be used for a specifically defined disease process and is constructed based on the current state of knowledge at the time it is developed. A nomenclature system differs fundamentally from categorization systems that use identifiable classification criteria or diagnostic criteria to decide what disease definition is fulfilled by an actual specific patient.
Methods During 2011, a group of 28 acknowledged experts in vasculitis from 12 countries convened and a modified Nominal Group Technique was used to discuss and decide on each issue. Each change or addition in a name or definition that arose from the group deliberations was posed to the group for a vote, followed by an additional two week on-line discussion period, and then a vote by all members. Greater than 80% agreement of the entire group was required to make a change in the previous names or definitions, or to add a new name or definition.
Results We retained the original concept of dividing the vasculitides according to the most prevalent vessel size being involved. The original list of 10 vasculitides was extended to include 27 diseases with vasculitis, and a new hierarchy was established. Moreover, some names were renamed either based on a wish to avoid eponymic names or in order to better mirror current knowledge on pathogenesis. The new names and abbreviations when available are listed below:
Large Vessel Vasculitis (LVV): Takayasu Arteritis (TAK) and Giant Cell Arteritis (GCA)
Medium Vessel Vasculitis (MVV): Polyarteritis Nodosa (PAN) and Kawasaki Disease (KD)
Small Vessel Vasculitis (SVV): ANCA-Associated Vasculitis (AAV) including: Microscopic Polyangiitis (MPA), Granulomatosis with Polyangiitis (Wegener’s) (GPA) and Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA) and Immune Complex SVV including: Anti-GBM Disease, Cryoglobulinemic Vasculitis, IgA Vasculitis (Henoch-Schönlein) (IgAV) and Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis) (HUV).
Variable Vessel Vasculitis (VVV): Behçet’s Disease (BD) and Cogan’s Syndrome (CS).
Single Organ Vasculitis (SOV): Cutaneous Leukocytoclastic Angiitis, Cutaneous Arteritis, Primary CNS Vasculitis and Isolated Aortitis.
Vasculitis Associated with Systemic Disease: Lupus Vasculitis, Rheumatoid Vasculitis and Sarcoid Vasculitis.
Vasculitis Associated with Probable Etiology: Hepatitis C Virus-Associated Cryoglobulinemic Vasculitis, Hepatitis B Virus-Associated Vasculitis, Syphilis-Associated Aortitis, Serum Sickness-Associated Immune Complex Vasculitis, Drug-Associated Immune Complex Vasculitis, Drug-Associated ANCA-Associated Vasculitis and Cancer-Associated Vasculitis.
Disease names and definitions evolve over time. The present nomenclature system is suggested to be more relevant and more valuable by including additional categories of vasculitis, and by adjusting names and definitions based on current trends in usage and on advances in the understanding of disease manifestations and mechanisms.
Disclosure of Interest None Declared
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