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We recently compared the annual incidence of primary systemic vasculitis (PSV) in two different regions of Europe (Norwich, UK (latitude 52°N) and Lugo, Spain (latitude 43°N)).1Wegener's granulomatosis (WG) was more common in Norwich (10.6/million) than in Spain (4.9/million), though the overall incidence of PSV was similar. This supports the idea that environmental factors may be important in the aetiopathogenesis of PSV. To extend our observations we have now studied the incidence of PSV in northern Europe (Tromsø, Norway (latitude 70°N)). The same methodology was used as in the previous study.1 All new patients presenting with PSV between 1 January 1988 and 31 December 1998 were identified in the three centres. WG, Churg-Strauss syndrome (CSS), and polyarteritis nodosa (PAN) were classified by the American College of Rheumatology (1990) criteria,2-4 and microscopic polyangiitis (MPA) and classical PAN by the Chapel Hill consensus definition.5 Incidence figures were calculated using the Poisson distribution for the observed number of cases.
Table 1 shows the results obtained. The overall incidence and pattern of vasculitis was similar in the three regions, but there were some differences. MPA was less common in Tromsø than in the other two regions, and there was a trend for WG to be more common in the north. CSS was more common in Norwich than in the other two regions. In all areas and all disease categories the incidence was greater in men than women and showed a peak incidence at age 65–74. Overall, WG is the most common type of PSV and classical PAN the rarest.
These results support the notion suggested by doctors interested in vasculitis that there are geographical differences in the incidence of WG, MPA, and CSS, and, in particular, there is an inverse relation between the incidence of WG and MPA. In clinical practice MPA and WG can be difficult to distinguish. Possibly, despite our best attempts to harmonise the application of classification criteria/definitions, there were still differences in approach. The reason for the apparent excess of CSS in Norwich is unclear but might reflect local environmental factors. The aetiopathogenesis of PSV is unknown, but both genetic and environmental factors are likely to be important. The clinically observed differences between MPA and WG may reflect interaction of varying trigger factors on a heterogeneous genetic background. It should therefore not be assumed that the same triggers operate in all regions of Europe.