Epidemiology of ANCA associated vasculitis

ANCA associated vasculitis (AAV) comprises three syndromes with systemic vasculitis (Wegener’s granu-lomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA)), which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies...

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Bibliographic Details
Main Authors: Wenche Koldingsnes, Hans C. Nossent
Other Authors: The Pennsylvania State University CiteSeerX Archives
Format: Text
Language:English
Subjects:
Online Access:http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.622.6867
http://www.ntnu.no/ojs/index.php/norepid/article/viewFile/62/52/
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Summary:ANCA associated vasculitis (AAV) comprises three syndromes with systemic vasculitis (Wegener’s granu-lomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA)), which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA). Polyarteritis nodosa (PAN) is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV. Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.