Page 94 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

70 David S. Younger

0.8 per million for the whole population. The point prevalence as of June 2012 was estimated
at 13.2 per million for the whole population.

Table 3. Global Incidence and Prevalence of TAK

Authors (References) Country Study Period Incidence per Prevalence
106 per 106
Watts et al. [52] UK 2000-2005 0.8 4.7
Mohammad et al. [53] Sweden 1997-2011 0.8 ..
Waern et al. [54] Sweden 1969-1976 0.8 6.4
Reinhold-Keller et al. [55] Germany 1998-2000 0.5 ..
Dreyer et al. [56] Demark 1990-2009 0.4 ..

The incidence findings were comparable with the reported incidence of 0.8, 0.5, and 0.4
per million respectively in previous studies from Sweden [54], Germany [55], and eastern
Denmark [56] although the prevalence of TAK was somewhat higher than the prevalence of
6.4 per million previously reported in Sweden [54].

Medium-Size Vessel Vasculitis

Polyarteritis Nodosa

Background
The ACR 1990 [57] and the Chapel Hill Consensus Conference (CHCC) [58] criteria for

PAN have been employed in the case definitions of most epidemiological studies as well as,
the criteria of other investigators [59, 60] for adult cases, and the criteria of the Turkish
Pediatric Vasculitis Study Group [61, 62] for pediatric cases, stratified into cutaneous and
classic PAN. Mahr and colleagues [60] defined polyarteritis nodosa (PAN) as a
predominantly medium-size vessel disease that occurs alone or in association with hepatitis B
virus (HBV) infection, and angiographically documented aneurysms or histological proof of
vessel inflammation, without glomerulonephritis, lung hemorrhage, or antinuclear cytoplasm
antibody (ANCA) positivity. This author was unable to find GWAS for PAN.

Epidemiology
The global incidence and prevalence of PAN is shown in Table 4. In a capture-recapture

study in the calendar year 2000 in Seine-St. Denis, a northeastern suburb of Paris with a
population of 1,093,515 adults, 28% of whom were of non-European ancestry, from among
general practitioners, departments of all of the public hospitals, two large private clinics, and
the National Health Insurance System. The prevalence of PAN was estimated at 30.7 per
million adults, however previous studies based upon the most restrictive and biopsy-
dependent Chapel Hill Consensus Conference (CHCC) [58] criteria estimated PAN to be 9
per million adults, in comparison to 33 per million based upon the less specific ACR criteria
[57] that fail to discriminate between MPA and PAN. The total prevalence estimate for all
disorders was 90.3 per million, which sub-stratified for geographic origin showed a two-fold
higher incidence rate for subjects of European than non-European ancestry respectively,
104.7 compared to 52.5 per million.

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