Page 91 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Epidemiology of Primary Systemic Vasculitis 67

Table 1. Classification of Primary Systemic Vasculitides

LARGE VESSEL VASCULITIS
Giant cell arteritis [formerly Temporal arteritis] (GCA)
Takayasu arteritis (TAK)
MEDIUM VESSEL VASCULITIS
Kawasaki Disease (KD)
Polyarteritis nodosa (PAN)
SMALL VESSEL VASCULITIS
Anti-neutrophil cytoplasmic antibody (ANCA)-Associated Vasculitis (AAV)
Granulomatosis with polyangiitis [formerly Wegener granulomatosis] (GPA)
Microscopic polyangiitis [formerly microscopic polyarteritis] (MPA)
Eosinophilic granulomatosis with polyangiitis [formerly Churg-Strauss syndrome] (EGPA)

Table 2. Global Incidence and Prevalence of GCA

Authors (References) Country Study Period Incidence Prevalence
per 106 per 106
Kobayashi et al. [11] Japan 1997 1.47 ..
Herlyn et al. [9] Germany 2006 2.71 171 per 106
Dunstan et al. [7] Australia 1992-2011 3.20 ..
Bas-Lando et al. [10] Israel 1980-2004 9.50 ..
Gonzalez-Gay et al. [15, 16] Spain 1981-2005 10.13 ..
Gonzalez-Gay et al. New Zealand 1996-2005 12.7 ..
Mohammad et al. [17] Sweden 1997-2010 13.3 ..
Salvarani et al. [19] US 1950 to 1999 18.8 ..
Smeeth et al. [18] UK 1990-2001 22.0 ..
Norway 1992-1996 27.50 ..
Haugeberg et al. [13, 14] Norway 1992-1996 36.70 ..
Norway 1992-1996 32.8 ..

The highest known incidence of GCA was reported by Haugeberg and colleagues [13,
14] without major differences between northern and western Norway compared to those of
Southern Norway. By comparison, very low incidence rates of GCA were found in Saudi
Arabian [21], Mexican [22], and Japanese populations [11]. Dustan and colleagues [7] found
evidence of seasonal variation in the incidence of GCA in Australian Bureau of Statistics
population data for South Australia noting higher rates in the summer months (p<0.015).
Abdul-Rahman and coworkers [12] noted a cyclic annual incidence with peaks five years
between 1996 and 2005, and a statistically insignificant (p<0.9), but suggestive seasonal
variation nonetheless, with more cases diagnosed in the spring than in summer, fall and
winter. Bas-Lando and coworkers [10] noted more common onset of GCA in the late spring
and early summer with fluctuation in the annual incidence that included three distinctive
peaks during the twenty-five year period of observation among 170 patients in Jerusalem
between 1980 and 2014. Ninan and coworkers [8] noted that the relative survival for different
followup periods in South Australian patients with GCA linked to birth, death and marriage
registry records showed similar mortality to the general population of age-matched and sex-
matched controls. According to Herlyn and colleagues [9] who studied inhabitants of the city
of Luebeck and the rural region of Segeberg in northern Germany, noted that GCA was the
most prevalent systemic vasculitis in 2006 with 171 per million inhabitants followed by

Complimentary Contributor Copy