Epidemiology of ANCA-associated Vasculitis

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AAV

There is a broad consensus that for primary, systemic, medium- and small-vessel vasculitis (including WG, CSS, PAN, and MPA) the overall annual incidence is approximately 10 to 20/million and the peak age of onset is 65 to 74 years (Table 1).8, 10, 11

WG

In 1936, Wegener first described a disease characterized by necrotizing granulomata of the upper and lower respiratory tract, focal glomerulonephritis, and necrotizing systemic vasculitis.12 The annual incidence of WG in the past decade has been estimated to be 8 to 10/million. WG is slightly more common in men than women.

MPA

The original description of periarteritis nodosa by Kussmaul and Maier was of a patient with inflammation and necrosis of medium-sized arteries leading to aneurysm formation and organ infarction. Davson and colleagues53 described patients with segmental necrotizing glomerulonephritis who also had features of PAN with extrarenal small and medium artery involvement. The term microscopic polyarteritis was used to describe these patients in whom the dominant feature was rapidly progressive renal

CSS

In 1951, Churg and Strauss described the post-mortem features of 13 patients who died following an illness characterized by asthma, eosinophilia, fever, systemic features, and granulomatous necrotizing vasculitis. Several studies have now included data on CSS; the incidence is generally reported to be in the range of 1.0 to 3.0/million (Table 7, Table 8). The highest figure was based on a single case seen during the period 1976 to 1979 in Olmsted County, MN.60 CSS is the least common of the

Summary

The epidemiology of the systemic vasculitides has been increasingly well documented in the past 20 years. There is an interesting age, geographic, and ethnic tropism that is gradually being revealed. Slow progress is being made on understanding the underlying causative factors but despite detailed and numerous studies no unequivocal environmental factors have emerged. The challenge still remains to the epidemiologist as improvements in classification methodology are promising a better

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