Page 101 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Epidemiology of Primary Systemic Vasculitis 77

Table 6. Global Incidence and Prevalence of AAV

Authors (References) Country Study Period Incidence per Prevalence
106 per 106
UK 1988-1992 8.71 --
6.82 --
UK 1988-1992 1.53 --
10.31 --
Watts et al. [102] UK 1988-1992 8.92 --
UK 1993-1997 3.73 --
8.81 64.31
UK 1993-1997 8.41 95.01
2.32 17.52
UK 1993-1997 5.02 39.12
2.33 11.73
1995-1999 2.23 22.33

2000-2004 -- 23.71

Ormerod et al. [103] Australia + UK 1995-1999 -- 25.12
2000-2004
-- 10.73
1995-1999
9.81 --
2000-2004 10.12 --
0.93 --
Seine-St. Denis 2000
County, Paris

Mahr et al. [60] Seine-St. Denis 2000
County, Paris

Seine-St. Denis 2000
County, Paris

Sweden 1997-2006

Mohammad et al. [64] Sweden 1997-2006

Sweden 1997-2006

1GPA; 2MPA; 3EGPA; 4Capital Territory; 5New South Wales.

In a comparison of primary systemic vasculitis in the Australian Capital Territory and
Southeastern New South Wales between 1995 and 1999, and between 2000 and 2004,
Ormerod and colleagues [103] noted similar disease-specific incidences for each of the two
periods, with 8.8 and 8.4 per million for GPA, 2.3 and 5.0 per million for MPA, and 2.3 and
2.2 per million for EGPA in the Australian Capital Territory compared to south-eastern New
South Wales with a trend for higher values in MPA and GPA in rural areas. A similar relation
was found in disease specific prevalence for each of the two periods, with 64.3 and 95.0 per
million for GPA, 17.5 and 39.1 per million for MPA, and 11.7 and 22.3 per million for EGPA
in the Australian Capital Territory compared to south-eastern New South Wales. In incident
cases of primary systemic vasculitis identified in Seine-St. Denis County, Paris, Mahr and
colleagues [60] estimated the prevalence of GPA 23.7, MPA 25.1, and EGPA 10.7 per
million adults in a population of 1,093,515, 28% of whom were of non-European ancestry,
with an overall prevalence that was two-fold higher for those of European ([104] 75 per
million) compared to others of non-European ancestry (52.5 per million). Mohammad and
colleagues [64] estimated incident cases of GPA, MPA and EGPA in southern Sweden
respectively, 9.8, 10.1, and 0.9 per million in a total population of 641,000 between 1997 and
2006, with a progressive increase in age-specific incidence rates over the study period.

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