Necrotizing glomerulonephritis is very common. Necrotizing arteritis involving small and medium arteries may be present. MPA is necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels. The change to GPA was influenced by a decision that had already been made by several relevant medical societies. The eponyms Wegener’s granulomatosis and Churg-Strauss syndrome were replaced with the more descriptive terms GPA and EGPA respectively. AAV is subdivided into microscopic polyangiitis (MPA), granulomatosis with polyangiitis (Wegener’s) (GPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). This is important because mounting evidence indicates that the ANCA specificity identifies distinct categories of disease. A prefix should be added to indicate ANCA reactivity, e.g. ĪAV is necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels, associated with MPO-ANCA or PR3-ANCA. This categorization was not in CHCC 1994 and reflects a greater level of confidence that ANCA are of pathogenic significance in AAV. SVV is divided into antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immune complex SVV. This was concluded after substantial debate and careful review of available research and clinical data to try to identify other distinguishing features. Thus, the major discriminator between TA and GCA is the age of the patient. GCA often involves the temporal artery, and onset is usually in patients older than 50 and often associated with polymyalgia rheumatica. GCA is arteritis, often granulomatous, usually affecting the aorta and its major branches, with a predilection for the branches of the carotid and vertebral arteries. Onset usually is in patients younger than 50. TA is arteritis, often granulomatous, predominantly affecting the aorta and its major branches. The two major variants of LVV are Takayasu arteritis (TA) and giant cell arteritis (GCA) For example, only a few branches of the carotid arteries may be affected when there is involvement of numerous small branches extending into the head and neck, for example small ocular and periocular arteries. This definition does not state that LVV predominantly affects large vessels because in many patients the number of medium and small arteries affected is greater than the number of large arteries affected. Hepatitis C virus–associated cryoglobulinemic vasculitisĭrug-associated immune complex vasculitisĭrug-associated ANCA-associated vasculitisĬHCC 2012 defines LVV as vasculitis affecting the aorta and its major branches more often than other vasculitides, however, any size artery may be affected. Vasculitis associated with probable etiology Vasculitis associated with systemic disease Primary central nervous system vasculitis Isolated aortitis Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis) Granulomatosis with polyangiitis (Wegener’s) (GPA)Įosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)Īnti-glomerular basement membrane (anti-GBM) disease Notable changes include the introduction of new terms such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and IgA vasculitis, and the inclusion of categories for variable vessel vasculitis and secondary forms of vasculitis.Īntineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) This overview summarizes the 2012 CHCC and points out the changes compared to the 1994 CHCC. The goals were to change names and definitions as appropriate, and add important categories of vasculitis not included in CHCC 1994. To address these issues, a second International Chapel Hill Consensus Conference was held in 2012. In addition, CHCC 1994 did not propose a nomenclature for some relatively common forms of vasculitis, such as vasculitis secondary to other diseases. Since then, there have been substantial advances in our understanding of vasculitis and changes in medical terminology. The first (1994) International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC) proposed names and definitions for the most common forms of vasculitis. This is problematic for both research on vasculitis as well as clinical care of patients with vasculitis. The nomenclature and classification of vasculitis has been difficult and controversial for many decades.
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