Epidemiology of Vasculitides
Vasculitides are rare diseases. There are only few epidemiological studies on vasculitides in adults in Canada, and only a few more have been done in the United States. However, based on European, Australian, New-Zealander and Japanese studies, one can estimate the overall prevalence and annual incidence ranges of primary vasculitides as follows:
| Vasculitis Type | Prevalence (per million) | Remarks | Annual Incidence (per million) | Remarks |
|---|---|---|---|---|
| GCA | 500-7,500 (of population >50 y-o) | Crude estimates (only few specific studies: 144 in Reunion isle in 2018 with biopsy +; 753 in Sweden with biopsy + in 2015) | 80-300 (of population >50 y-o) | Down to 5 in Israel in late 1960s; 27 in Reunion isle 2005-2018; up to 370 in Norway in mid 1990s; 20 in subjects <60 and up to 520 in those >80 y-o in Minnesota; 79 in Paris in 2017 |
| Takayasu | 4 to 8 | 4.7 to 7 in the UK in early 2000s; Up to 40 in Japan (no epidemiological data for India, but probably at least the same) | 1 to 2 | Down to 0.4 in Germany; Up to 2.6 in Minnesota in late 1970s, and 3.3 in Kuwait |
| PAN | 6 to 30 | Up to >30 in late 1990s before HBV-related PAN almost disappeared | 0.9-6.8 | Up to 8 in the UK, 16 in Kuwait in the late 1990s, and 77 in Alaska in late 1980s (HBV endemy) |
| Kawasaki | – | Acute disease (in general, but damage) | 90-500 (of children <5 y-o) | Down to 16 in Czech republic in late 1990s; Up to 2,180 in Japan; in US, 91 in Caucasian vs. 320 in Asian children in early 2000s |
| GPA | 50-250 | Down to 23 in Paris in 2000, 30 in NYC in 1990; Up to 135 in the UK in 2013, 160 in Sweden in 2007, 201 in Germany in 2013-2016, and 260 in Norway in 2013-2015 | 5 to 30 | Down to almost 0 in Japan, 2.9 in Spain; Up to 11 in Australia and Minnesota; 14 in adults in the UK between 2000-2013, 15.6 in Norway (1999-2013), and 34 in Germany in 2013-2016 |
| MPA | 25-150 | Down to 25 In Paris in 2000; Up to 160 in Norway in 2015 | 5 to 15 | Down to 2.7 In Germany in 2000s; up to 15 in Japan, and 24 in Kuwait, and 13 in Germany 2013-2016 |
| EGPA | 10 to 40 | Down to 7 in Germany in 1994; Up to 22 in Australia in 2004, and 42 in Norway in 2015 | 1 to 2 | Down to 0 in Japan; Up to 2.7 in the UK in late 1990s, 2.7 in Norway in 1999-2013, and 4 in Minnesota in late 1970s |
| Behçet | 10-500 | Extremely wide ethnic variations (from 6 in the UK in late 1970s to 4,200 in Turkey in 2000); 100-300 in US, mainly in immigrants; 24 in European-descent vs. 175 in Asian-descent vs 350 in North-African-descent French population | – | No precise estimation (chronic disease and wide geographical differences); around 4 in Minnesota in mid 2000s |
| CNS-V | – | No data | 2.4 | Only 1 study, in Minnesota |