Table 1.

Annual incidence of primary systemic vasculitis in different regions in Europe and the Middle Easta

DiseaseNorway (Tromsø)North Germany (Schleswig-Holstein)United Kingdom (Norwich)South Germany (Baden-Württemberg)Spain (Lugo)Kuwait (Kuwait City and Al-Jahra)
WG10.5 (7.6 to 14.2)8 (2 to 14)10.6 (7.8 to 14.0)6 (3 to 9)4.9 (2.4 to 8.8)0
MPA2.7 (1.3 to 4.8)3 (0 to 6)8.4 (5.9 to 11.5)2 (0 to 4)11.6 (7.6 to 17.0)25
PAN0.5 (0.06 to 1.8)1 (0 to 3)0.0 (0.0 to 0.8)2 (0 to 4)0.9 (0.1 to 3.2)16
CSS0.5 (0.06 to 1.8)0 (0 to 0)3.1 (1.7 to 5.2)1 (0 to 2)0.9 (0.1 to 3.2)NR
Total13.7 (10.3 to 17.8)12 (2 to 23)18.9 (15.1 to 23.4)11 (3 to 19)18.3 (13.1 to 24.8)47
  • a Adapted from references (16–18). Data are cases per million (95% confidence interval). Columns from left to right represent regions from north to south. Patients’ diseases were diagnosed according to the criteria of the Chapel Hill Consensus Conference. Incidence numbers of Norway, the United Kingdom, and Spain are the average of the period between January 1, 1988, and December 31, 1998. Numbers from Germany are an average of the period between January 1, 1998, and December 32, 2002, whereas in Kuwait, the period between January 1, 1993, and December 31, 1996, was analyzed. CSS, Churg-Strauss syndrome; MPA, microscopic polyangiitis; NR, not reported; PAN, polyarteritis nodosa; WG, Wegener's granulomatosis; Total annual incidences of all diseases together.