New Nomenclature on the Vasculitides

I always look forward to the case report sessions during PRA Convention. It’s always interesting how difficult cases (either diagnostically or management-wise) turn out. However, one particular case caught my attention. Initially, I thought there were new vasculitic syndromes to study. But after conferring with some colleagues, it turned out that they were just new names to old diseases. A quick check with PubMed showed that a paper on the new nomenclature (the 2012 Chapel Hill Consensus Conference system) was published just last January 2013.

The 2012 CHCC is a nomenclature system that seeks to improve on the previous 1994 definitions by adopting names and definitions that are widely accepted and adding important categories of vasculitis that were not previously included. An interesting move was to use less eponyms in the new system particularly when there are descriptive terms that offered better understanding of the disease’s pathophysiology.

Likewise, a better definition of vessel involvement was used in the new system. Large vessel vasculitis (LVV) affects the aorta, its major branches and/or analogous veins. Medium vessel vasculitis (MVV) involves the main visceral arteries, veins and their initial branches. While small vessel vasculitis (SVV) affects intra-parenchymal arteries, arterioles, capillaries, venules and veins. Introduced were the variable vessel vasculitis (VVV) that have no dominant type of vessel involved and can affect vessels of any size and type. And single organ vasculitis (SOV) affecting arteries or veins of any size in a single organ that has no features to indicate that it is a limited expression of a systemic vasculitis.

Below is a listing of the new vasculitis nomenclature (and accepted abbreviations):

  • Large vessel vasculitis

    • Takayasu arteritis (TAK) – no ‘s

    • Giant cell arteritis (GCA) – previously known as temporal arteritis

  • Medium vessel vasculitis

    • Polyarteritis Nodosa (PAN)

    • Kawasaki disease (KD) – again, no ‘s

  • Small vessel vasculitis

    • ANCA-associated vasculitis (a.k.a. pauci-immune)

      • Microscopic polyangiitis (MPA)

      • Granulomatosis with polyangiitis (GPA) – new name for Wegener granulomatosis

      • Eosinophilic granulomatosis with polyangiitis (EGPA) – new name for Churg Strauss Syndrome

    • Immune complex vasculitis

      • Anti-GBM disease

      • Cryoglobulinemic Vasculitis (CV)

      • IgA Vasculitis (IgAV) – new name for Henoch Schonlein Purpura

      • Hypocomplementemic urticarial vasculitis (HUV) – instead of simply urticarial vasculitis – a.k.a. anti-C1q vasculitis

  • Variable vessel vasculitis

    • Behcet’s Disease (BD)

    • Cogan’s Syndrome (CS)

  • Single organ vasculitis

    • Cutaneous leucocytoclastic angiitis – previously this was placed under SVV

    • Cutaneous arteritis

    • Primary CNS vasculitis

    • Isolated aortitis

  • Vasculitis associated with probable etiology

    • Hepatitis C virus associated cryoglobulinemic vasculitis

    • Hepatitis B virus associated vasculitis

    • Syphilis associated aortitis

    • Drug associated immune complex vasculitis

    • Drug associated ANCA associated vasculitis

    • Cancer-associated vasculitis

I opted to simply list down the new names and leave it to the reader to explore the definitions. It’s already difficult studying vasculitis and the new nomenclature is bound to create some confusion. But as any student of medicine knows, the body of knowledge is forever changing.

Reference: Jennette JC, Falk RJ, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arth & Rheum 2013; 65(1): 1-11.

Author: Sids Manahan MD 🇵🇭

Rheumatology. Internal Medicine. Educating Patients and Colleagues. Curating Rheumatology. Bloggero-Wanabe.

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