Page 95 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Epidemiology of Primary Systemic Vasculitis 71

Table 4. Global Incidence and Prevalence of PAN

Authors (References) Country Study Period Incidence per Prevalence
106 per 106
Jennette et al. [58] US 2012 -- 30.7
Lightfoot et al. [57] US 1990 -- 9.0
Mohammad et al. [64] Sweden 1997-2006 0.9 --
UK1 1988-1998 9.7 --
Watts et al. [65] Spain2 1988-1998 6.2 --
UK1 + Spain2 1995-1999 2.3 --
Omerod and Cook [66] UK1 + Spain2 2000-2004 1.1 --
1Norwich.
2Lugo.

Not more than 30% of the PAN cases appeared to be HBV-related with most diagnosed
during the 1980?s suggesting that the reported incidence of HBV-associated PAN of 77 per
million so noted in a small population of Alaskan Eskimos with high rates of HBV infection
[63] was currently decreasing as a consequence of vaccination campaigns and the improved
safety of blood products. Mohammad and colleagues [64] studied incident cases of PAN,
GPA, MPA, and EGPA in two health care districts of South Sweden of central and southwest
Skâne containing fourteen municipalities with a population of 641,763 for the period 1997 to
2006 from hospital databases identifying 144 cases of primary systemic vasculitis, of which 6
were PAN. The annual incidence rates was 21.8 per million for all patients, and 0.9 per
million for PAN. Watts and colleagues [65] studied incident cases of PAN, GPA, MPA, and
EGPA in two regions of Europe, among general medical practices of the Norwich Health
Authority (NHA) in Norfolk, UK covering 413,500 patients; and in the referral centre of
Lugo, Spain at the Hospital Xeral-Calde, with a population of 250,000 people between 1988
and 1998. The study employed the 2012 Revised CHCC [58] and ACR criteria [57] noting
an overall incidence of primary systemic vasculitis that was 18.9 Norwich compared to 18.3
per million in Spain, with a higher incidence of PAN in Norwich than in Spain, 9.7 versus 6.2
per million respectively. Omerod and colleagues [66] studied the prevalence and incidence
for primary systemic vasculitides for the two five year periods of 1995 to 1999, and 2000 to
2004, in the Australian Capital Territory and the surrounding rural regions.

Altogether, 41 cases of primary systemic vasculitides including PAN, GPA, MPA
and EGPA were identified between 1995 and 1999, and 67 between 2000 and 2004.
This yielded a prevalence of 95 and 148 per million, with a similar annual incidence of 17 per
million for Norwich, UK and Lugo, Spain; and a disease-specific incidences for PAN of 2.2
and 1.1 for the two successive periods.

Kawasaki Disease

Background
Kawasaki Disease (KD), or mucocutaneous lymph node syndrome, is an acute, self-

limited systemic vasculitis of medium- and small-sized vessels occurring predominantly in
children age 6 months to 5 years [67]. It is the second commonest childhood vasculitis and
the leading cause of acquired childhood heart disease in developed countries [68, 69]. The
distribution is worldwide with an incidence in Japanese populations 10 to 15-fold greater than

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