Epidemiology

​There is a paucity of robust epidemiological data on adult-onset Still’s disease (AOSD), with very few prospectively collected data. It is a rare disease with an annual incidence that varies in different studies between 0.16 per 100,000 and 0.4 per 100,000 and is associated with a clearly increased mortality.[1][2][4]​​ The estimated point prevalence was reported by one review at 0.73-6.77 per 100,000 and by another at between 1 and 34 cases per million.[1][5]​​ Incidence and prevalence are higher in recent studies compared with older studies, likely because of increased awareness.[5]

Most cases occur in young adults, with a bimodal pattern showing two peaks of onset at aged 16-25 years and 36-46 years.[1][4][5]​​​ However, there is growing evidence of a further peak at aged 60-65 years, although delayed diagnosis may be a contributory factor.[5][7][8]​​​​[9][10] 

Most studies have noted a female predominance, with women accounting for 60% to 80% of cases, although a minority have reported an equal distribution between men and women.[7][8][10]​​[11]​​[12]​​​ ​​​​

In one of the largest nationwide studies of AOSD epidemiology in the US, analysis of 5-year retrospective data between 2009 and 2013 revealed an inpatient mortality of 2.6%.[12]

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